Background：One-lung ventilation （OLV） is a common ventilation technology during thoracic surgery that can cause serious clinical problems.We aimed to conduct a meta-analysis to compare oxygenation and intrapulmonar...Background：One-lung ventilation （OLV） is a common ventilation technology during thoracic surgery that can cause serious clinical problems.We aimed to conduct a meta-analysis to compare oxygenation and intrapulmonary shunt during OLV in adults undergoing thoracic surgery with dexmedetomidine （Dex） versus placebo to assess the influence and safety of using Dex.Methods：Randomized controlled trials comparing lung protection in patients who underwent thoracic surgery with Dex or a placebo were retrieved from PubMed,EMBASE,MEDLINE,Cochrane Library,and China CNKI database.The following information was extracted from the paper：arterial oxygen partial pressure （PaO2）,PaO2/inspired oxygen concentration （PaO2/FiO2,oxygenation index [OI]）,intrapulmonary shunt （calculated as Qs/Qt）,mean arterial pressure （MAP）,heart rate （HR）,tumor necrosis factor-α （TNF-α）,interleukin （IL）-6,superoxide dismutase （SOD）,and malondialdehyde （MDA）.Results：Fourteen randomized controlled trials were included containing a total of 625 patients.Compared with placebo group,Dex significantly increased PaO2/FiO2 （standard mean difference [SMD] =0.98,95% confidence interval [CI] [0.72,1.23],P 〈 0.00001）.Besides,Qs/Qt （SMD=-1.22,95% CI [-2.20,-0.23],P =0.020）,HR （SMD=-0.69,95% CI [-1.20,0.17],P =0.009）,MAP （SMD=-0.44,95% CI [-0.84,0.04],P =0.030）,the concentrations ofTNF-α （SMD =-1.55,95% CI [-2.16,-0.95],P 〈0.001）,and IL-6 （SMD =-1.53,95% CI [-2.37,-0.70],P =0.0003） were decreased in the treated group,when compared to placebo group.No significant difference was found in MDA （SMD =-1.14,95% CI [-3.48,1.20],P =0.340） and SOD （SMD =0.41,95% CI [-0.29,1.10],P =0.250） between the Dex group and the placebo group.Funnel plots did not detect any significant publication bias.Conclusions：Dex may improve OI and reduce intrapulmonary shunt during OLV in adults undergoing thoracic surgery.However,this conclusion might be weakened by the limited number of pooled studies and p展开更多
BACKGROUND: Pulmonary complications after orthoto- pic liver transplantation (OLT) include high morbidity and mortality. Experimental data have suggested hepatic ische- mia and reperfusion are induced by pro-inflammat...BACKGROUND: Pulmonary complications after orthoto- pic liver transplantation (OLT) include high morbidity and mortality. Experimental data have suggested hepatic ische- mia and reperfusion are induced by pro-inflammatory cyto- kines. The high level of inflammatory cytokines might ad- ditionally influence pulmonary cappillary fluid filtration. The objectives of this study were to measure the concentra- tions of tumor necrotic factor-alpha (TNF-α), interleukin- 6 (IL-6) and interleukin-8 (IL-8) during OLT and to in- vestigate the relationship between these cytokines and post- operative pulmonary complications. METHODS: Twenty-two patients undergoing OLT were divided into two groups according to whether they had postoperative pulmonary complications: group A consis- ting of 8 patients with postoperative pulmonary complica- tions , and group B consisting of 14 patients without post- operative pulmonary complications. Enzyme-linked im- munoassay (ELISA) was used to determine serum TNF-α, IL-6 and IL-8. Blood samples were taken at the beginning of operation (T0 ), clamping and cross-clamping of the in- ferior cava and portal vein (T1, T2 ), 90 minutes and 3 hours after reperfusion (T3 , T4 ) and 24 hours after opera- tion (T5). RESULTS: The level of PaO2/FiO2 in group A was lower than that in group B ( P <0. 05 ). The concentrations of TNF-α, IL-6 and IL-8 in the two groups increased rapidly at T2 , peaked at T3 , decreased rapidly after T3 until 24 hours after operation. The concentrations of TNF-α, IL-6 and IL-8 in group A were higher than those in group B at T2, T3, and T4(P<0.05). CONCLUSION: After un-clamping of the inferior cava and portal vein, the serum concentrations of TNF-α, IL-6 and IL-8 increased may be related to pulmonary injury after he- patic ischemic reperfusion.展开更多
By using the observation data from 89 weather stations in Xinjiang during 1961-2010, this paper analyzed the basic climatic elements including temperature, precipitation, wind speed, sunshine duration, water vapor pre...By using the observation data from 89 weather stations in Xinjiang during 1961-2010, this paper analyzed the basic climatic elements including temperature, precipitation, wind speed, sunshine duration, water vapor pressure, and dust storm in the entire Xinjiang and the subareas: North Xinjiang, Tianshan Mountains, and South Xinjiang. The results indicate that from 1961 to 2010 the annual and seasonal mean temperatures in the entire Xinjiang show an increasing trend with the increasing rate rising from south to north. The increasing rate of annual mean minimum temperature is over twice more than that of the annual mean maximum temperature, contributing much to the increase in the annual averages. The magnitude of the decrease rate of low-temperature days is larger than the increase rate of high-temperature days. The increase of warm days and warm nights and the decrease of cold days and cold nights further reveal that the temperature increasing in Xinjiang is higher. In addition, annual and seasonal rainfalls have been increasing. South Xinjiang experiences higher increase in rainfall amounts than North Xinjiang and Tianshan Mountains. Annual rainy days, longest consecutive rainy days, the daily maximum precipitation and extreme precipitation events, annual torrential rain days and amount, annual blizzard days and amount, all show an increasing trend, corresponding to the increasing in annual mean water vapor pressure. This result shows that the humidity has increased with temperature increasing in the past 50 years. The decrease in annual mean wind speed and gale days lessen the impact of dust storm, sandstorm, and floating dust events. The increase in annual rainy days is the cause of the decrease in annual sunshine duration, while the increase in spring sunshine duration corresponds with the decrease in dust weather. Therefore, the increase in precipitation indicators, the decrease in gales and dust weather, and the increasing in sunshine duration in spring will be beneficial to crops growth.展开更多
The relationship between the amount of tea cream and the chemical components contents in the original green tea infusion was investigated. The results showed that the amounts of tea cream in the green tea infusions ob...The relationship between the amount of tea cream and the chemical components contents in the original green tea infusion was investigated. The results showed that the amounts of tea cream in the green tea infusions obtained from different cultivars and different parts of new shoots were varied. There were many chemical components participating in the formation of green tea cream. However, there were only the contents of caffeine （Y=0.85, P〈0.01） and polyphenols （Y=0.65, P〈0.05） in the original green tea infusion highly correlated with the amount of green tea cream. Stepwise regression analysis of overall chemical components indicated that the contents of caffeine and gallated catechins in the original green tea infusion had a significant effect （P〈0.01） on green tea cream levels. Cream （g L-1）=-172.071＋ 0.129×Ccaffeine＋0.024×Ggallated catechins （R2=0.936）. The amount of green tea cream can be predicted by the contents of gallated catechins and caffeine in the original tea infusion. Principal component analysis also indicated that catechins, minerals, and polysaccharides were the important chemical components in the formation of green tea cream.展开更多
BACKGROUND With the increasingly extensive application of artificial intelligence(AI)in medical systems,the accuracy of AI in medical diagnosis in the real world deserves attention and objective evaluation.AIM To inve...BACKGROUND With the increasingly extensive application of artificial intelligence(AI)in medical systems,the accuracy of AI in medical diagnosis in the real world deserves attention and objective evaluation.AIM To investigate the accuracy of AI diagnostic software(Shukun)in assessing ischemic penumbra/core infarction in acute ischemic stroke patients due to large vessel occlusion.METHODS From November 2021 to March 2022,consecutive acute stroke patients with large vessel occlusion who underwent mechanical thrombectomy(MT)post-Shukun AI penumbra assessment were included.Computed tomography angiography(CTA)and perfusion exams were analyzed by AI,reviewed by senior neurointerventional experts.In the case of divergences among the three experts,discussions were held to reach a final conclusion.When the results of AI were inconsistent with the neurointerventional experts’diagnosis,the diagnosis by AI was considered inaccurate.RESULTS A total of 22 patients were included in the study.The vascular recanalization rate was 90.9%,and 63.6%of patients had modified Rankin scale scores of 0-2 at the 3-month follow-up.The computed tomography(CT)perfusion diagnosis by Shukun(AI)was confirmed to be invalid in 3 patients(inaccuracy rate:13.6%).CONCLUSION AI(Shukun)has limits in assessing ischemic penumbra.Integrating clinical and imaging data(CT,CTA,and even magnetic resonance imaging)is crucial for MT decision-making.展开更多
Interferons(IFNs)play an important role in immunomodulatory and antiviral functions.IFN-induced necroptosis has been reported in cells deficient in receptor-interacting protein kinase 1(RIPK1),Fas-associated protein w...Interferons(IFNs)play an important role in immunomodulatory and antiviral functions.IFN-induced necroptosis has been reported in cells deficient in receptor-interacting protein kinase 1(RIPK1),Fas-associated protein with death domain(FADD),or caspase-8,but the mechanism is largely unknown.Here,we report that the DNA-dependent activator of IFN regulatory factors(ZBP1,also known as DAI)is required for both type Ⅰ(β)and type Ⅱ(γ)IFN-induced necroptosis.We show that L929 fibroblast cells became susceptible to IFN-induced necroptosis when RIPK1,FADD,or Caspase-8 was genetically deleted,confirming the antinecroptotic role of these proteins in IFN signaling.We found that the pronecroptotic signal from IFN stimulation depends on new protein synthesis and identified ZBP1,an IFN-stimulated gene(ISG)product,as the de novo synthesized protein that triggers necroptosis in IFN-stimulated cells.The N-terminal domain(ND)of ZBP1 is important for ZBP1–ZBP1 homointeraction,and its RHIM domain in the C-terminal region interacts with RIPK3 to initiate RIPK3-dependent necroptosis.The antinecroptotic function of RIPK1,FADD,and caspase-8 in IFN-treated cells is most likely executed by caspase-8-mediated cleavage of RIPK3,since the inhibitory effect on necroptosis was eliminated when the caspase-8 cleavage site in RIPK3 was mutated.ZBP1-mediated necroptosis in IFN-treated cells is likely physiologically relevant,as ZBP1 KO mice were significantly protected against acute systemic inflammatory response syndrome(SIRS)induced by TNF+IFN-γ.展开更多
Background：Dysferlinopathy is caused by mutations in the dysferlin （DYSF） gene.Here,we described the genetic features of a large cohort of Chinese patients with this disease.Methods：Eighty-nine index patients were...Background：Dysferlinopathy is caused by mutations in the dysferlin （DYSF） gene.Here,we described the genetic features of a large cohort of Chinese patients with this disease.Methods：Eighty-nine index patients were included in the study.DYSF gene analysis was performed by Sanger sequencing in 41 patients and targeted next generation sequencing （NGS） in 48 patients.Multiplex ligation-dependent probe amplification （MLPA） was performed to detect exon duplication/deletion in patients with only one pathogenic mutation.Results：Among the 89 index patients,79 patients were demonstrated to carry two disease-causing （73 cases） or possibly disease-causing mutations （6 cases）,including 26 patients with homozygous mutations.We identified 105 different mutations,including 59 novel ones.Notably,in 13 patients in whom only one pathogenic mutation was initially found by Sanger sequencing or NGS,3 were further identified to carry exon deletions by MLPA.The mutations identified in this study appeared to cluster in the N-terminal region.Mutation types included missense mutations （30.06%）,nonsense mutations （1 7.18%）,frameshift mutations （30.67%）,in-frame deletions （2.45%）,intronic mutations （17.79%）,and exonic rearrangement （1.84%）.No genotype-phenotype correlation was identified.Conclusions：DYSF mutations in Chinese patients clustered in the N-terminal region of the gene.Exonic rearrangements were found in 23% of patients with only one pathogenic mutation identified by Sanger sequencing or NGS.The novel mutations found in this study greatly expanded the mutational spectrum of dysferlinopathy.展开更多
This study aimed to investigate the effects of male hepatitis B virus(HBV)infection on male fertility,embryonic development,and in vitro fertilization/intracytoplasmic sperm injection(IVF/ICSI)outcomes.We performed a ...This study aimed to investigate the effects of male hepatitis B virus(HBV)infection on male fertility,embryonic development,and in vitro fertilization/intracytoplasmic sperm injection(IVF/ICSI)outcomes.We performed a retrospective cohort study that included 3965 infertile couples who received fresh embryo transfer cycles for the first time at the Fujian Maternity and Child Health Hospital(Fuzhou,China)from January 2018 to January 2021.Infertile couples were categorized based on their HBV infection status into the HBV group(HBV-positive men and HBV-negative women)and the control group(HBV-negative couples).A 1:1 propensity score matching was performed with relatively balanced covariates.Baseline characteristics,semen parameters,laboratory outcomes,clinical outcomes,and obstetric and neonatal outcomes were compared between groups.After propensity score matching,821 couples were included in each group.Both groups had similar semen parameters and obstetric and neonatal outcomes.The HBV group showed a significantly lower live birth rate than the control group(P<0.05).The HBV group had a significantly higher abortion rate than the control group(P<0.05).The rates of high-quality embryos and blastocyst formation were significantly lower in the HBV group than those in the control group(both P<0.05).In conclusion,in couples who undergo IVF/ICSI,male HBV infection reduces the live birth rate and increases the risk of miscarriage.However,the incidence of low birth weight in women with IVF/ICSI does not increase with male HBV infection.展开更多
BACKGROUND Severe acute pancreatitis(AP)is one of the most common diseases of the gastrointestinal tract and carries a significant financial burden with high disability and mortality.There are no effective drugs in th...BACKGROUND Severe acute pancreatitis(AP)is one of the most common diseases of the gastrointestinal tract and carries a significant financial burden with high disability and mortality.There are no effective drugs in the clinical management of severe AP,and there is an absence of evidence-based medicine concerning the treatment of severe AP.AIM To explore whether ulinastatin(UTI)can improve the outcome of severe AP.METHODS The present research included patients who were hospitalized in intensive critical care units(ICUs)after being diagnosed with severe AP.Patients received UTI(400000 IU)or placebos utilizing computer-based random sequencing(in a 1:1 ratio).The primary outcome measures were 7-d mortality,clinical efficacy,inflammatory response,coagulation function,infection,liver function,renal function,and drug-related adverse effects were evaluated.RESULTS A total of 181 individuals were classified into two groups,namely,the placebo group(n=90)and the UTI group(n=91).There were no statistically significant differences in baseline clinical data between the two groups.The 7-d mortality and clinical efficacy in the UTI group were remarkably improved compared with those in the placebo group.UTI can protect against hyperinflammation and improve coagulation dysfunction,infection,liver function,and renal function.UTI patients had markedly decreased hospital stays and hospitalization expenditures compared with the placebo group.CONCLUSION The findings from the present research indicated that UTI can improve the clinical outcomes of patients with severe AP and has fewer adverse reactions.展开更多
Microbiota in the gastrointestinal tract （GIT） of piglets during weaning transition can experience a sharp change which could result in growth reduction and diarrhea of weaned piglets. Dietary manipulations can play...Microbiota in the gastrointestinal tract （GIT） of piglets during weaning transition can experience a sharp change which could result in growth reduction and diarrhea of weaned piglets. Dietary manipulations can play an important role in attenuating such changes caused by weaning stress. Therefore, ileal and colonic contents of weaned piglets were used as inocula, mannan oligosaccharide （MOS） or sugar beet pulp （SBP） was supplied as single energy sources to investigate effects of MOS or SBP on the shifts of gastro-intestinal microflora and lactobacilli populations. The universal bacteria- and lactobacilli-specific PCR/denaturing gradient gel electrophoresis （DGGE）, cloning and sequencing techniques were used. DGGE profiles of the universal bacteria showed that great changes were found in the position, numbers and intensity of dominant bands after fermentation. The similarity of bacterial community between ileum and colon was increased to 85-97% by MOS or SBP treatment after fermentation from the similarity with 20% before fermentation. MOS treatment significantly increased the bacterial diversity and band number in both ileal and colonic fermentation （P〈0.05）. SBP treatment significantly increased the bacterial diversity and band number in colon （P〈0.05）. It implies that some species were enriched by the addition of MOS or SBP to increase the similarity and diversity of bacterial community in weaned piglets. Five specific bands appearing in MOS or SBP treatment group after fermentation were cloned and sequenced, the changes of species related to Prevotella and Ruminococcus were observed. Two bands related to uncultured bacterium with 98% similarity were detected by MOS or SBP treatment. However, there were no effects on the similarity, diversity index and lactobacilli species revealed by MOS or SBP treatment. These results imply that MOS or SBP could have beneficial effects on the weaning piglets by stablizing microbiota in the GIT microflora.展开更多
The atom-economical C-F insertion chemistry is emerged as a promising technology for the synthesis of various fluorinated scaffolds,which have wide applications both in the academic and the industrial com-munities.The...The atom-economical C-F insertion chemistry is emerged as a promising technology for the synthesis of various fluorinated scaffolds,which have wide applications both in the academic and the industrial com-munities.The past three years have witnessed rapid developments in this field.This highlight provides an overview on the evolution according to the fluorinating agents used.展开更多
In an attempt to explore the relationships between phosphatidylglycerol （PG） molecular species of thylakoid membrane lipids and sensitivities to chilling-induced photoinhibition, PG molecular species, D1 protein, el...In an attempt to explore the relationships between phosphatidylglycerol （PG） molecular species of thylakoid membrane lipids and sensitivities to chilling-induced photoinhibition, PG molecular species, D1 protein, electron transport activities of thylakoid membrane and the potential quantum yield （FvlFm） in rice treated under middle and low photon flux density （PFD） at 11℃ were analyzed by high performance liquid chromatography, enzyme hydrolysis, gas phase chromatography （GC） and so on. Results showed that the major molecular species of PGs in rice thylakoid membrane were 18：3/16：0, 18：3/16：1（3t）, 18：2/16：0, 18：2/16：1（3t）, 18：1/16：0, 18：1/16：1（3t）, 16：0/16：0, 16：0/16：1（3t）. There were large differences in the contents of unsaturated PG molecular species such as 18：1-3/16：0-16：1（3t） and saturated PG molecular species like 16：0/16：0-16：1（3t） among japonica cv 9516 0-9516）, japonica-indica hybrid F1 j-9516/i-SY63 （ji-95SY） and indica cv Shanyou 63 （i-SY63）. J-9516 containing higher contents of unsaturated PG molecular species was manifest in stable D1 protein contents under chill and tolerant to chill-induced photoinhibition. In contrast to j-9516, i-SY63 with lower contents of unsaturated PG molecular species, exhibited unstable D1 protein contents under chill and was sensitive to chill-induced photoinhibition, ji-95SY containing middle contents of unsaturated PG molecular species between those of j-9516 and i-SY63, exhibited mid extent of sensitivity to chill-induced photoinhibition. The losses in D1 protein also account for the inhibition in electron transport activity of thylakoid membrane and the observed decline in FvlFm. The PG molecular species that is efficient in raising chilling-resistant capacity were those containing unsaturated fatty acids, namely, unsaturated PG molecular species. These results implied that the substrate selectivity of the glycerol-3-phosphate acyltransferase in chloroplasts towards 16：0 展开更多
Temperature is one of the abiotic factors limiting growth and productivity of plants. In the present work, the effect of low non-freezing temperature, as an inducer of ＂chilling resistance＂, was studied in three cul...Temperature is one of the abiotic factors limiting growth and productivity of plants. In the present work, the effect of low non-freezing temperature, as an inducer of ＂chilling resistance＂, was studied in three cultivars of rice （Oryza sativa L.）, japonica cv. 9516 （j-9516）, the two parental lines of superhigh-yield hybrid rice between subspecies, Peiai/E32 （ji-PE）, and the traditional indica hybrid rice Shanyou 63 （i-SY63）. Leaves of chill-treated rice showed chilling-induced resistance, as an increase of their low-temperature tolerance was measured using chlorophyll fluorescence measurements, revealing a change in photosystem II （PSII） efficiency. After 5 d of exposure to 11 ~C under low light （100 pmol·m^-2·s^-1）, levels of unsaturated fatty acids in PSII thylakoid membrane lipids decreased during the initial 1-2 d, then increased slowly and reached 99.2%, 95.3% and 90.1% of the initial value （0 d） in j-9516, ji-PE and i-SY63, respectively, on the fifth day. However, under medium light （600 μmol·m^-2·s^-1）, all cultivars experienced similar substantial photoinhibition, which approached steady state levels after a decline in levels of unsaturated fatty acids in PSII thylakoid membrane lipids to about 57.1%, 53.8% and 44.5% of the initial values （0 d） in j-9516, ji-PE and i-SY63 on the fifth day. Under either chilling-induced resistance （the former） or low temperature photoinhibition （the latter） conditions, the changes of other physiological parameters such as D1 protein contents, electron transport activities of PSII （ETA）, Fv/Fm, xanthophyl cycle activities expressed by DES （deepoxide state） were consistent with that of levels of unsaturated fatty acids in PSII thylakoid membrane lipids. So there were negative correlations between saturated levels of fatty acids （16：1（3t）, 16：0, 18：0）, especially the 16：1（3t） fatty acid on thylakoid membrane and other physiological parameters, such as D1 protein contents, ETA and （A＋Z）/（展开更多
Background Mutations of the LMNA gene encoding lamin A and C are associated with dilated cardiomyopathy （DCM）, conduction system defects and skeletal muscle dystrophy. Here we report a family with a mutation of the ...Background Mutations of the LMNA gene encoding lamin A and C are associated with dilated cardiomyopathy （DCM）, conduction system defects and skeletal muscle dystrophy. Here we report a family with a mutation of the LMNA gene to identify the relationship between genotype and phenotype. Methods All 30 members of the family underwent clinical and genetic evaluation. A mutation analysis of the LMNA gene was performed. All of the 12 exons of LMNA gene were extended with polymerase chain reaction （PCR） and the PCR products were screened for gene mutation by direct sequencing. Results Ten members of the family had limb-girdle muscular dystrophy （LGMD） and 6 are still alive. Two patients suffered from DCM. Cardiac arrhythmias included atrioventricular block and atrial fibrillation; sudden death occurred in 2 patients. The pattern of inheritance was autosomal dominant. Mutation c.73C〉G （R25G） in exon 1 encoding the globular domains was confirmed in all of the affected members, resulting in the conversion of arginine （Arg） to glycine （Gly）. Conclusions The mutation R25G in exon 1 of LMNA gene we reported here in a Chinese family had a phenotype of malignant arrhythmia and mild LGMD, suggesting that patients with familial DCM, conduction system defects and skeletal muscle dystrophy should be screened by genetic testing for the LMNA gene.展开更多
Substrate selectivity of glycerol-3-phosphate acyltransferase （EC 2. 3. 1. 15） of rice （Oryza sativa L.） was explored in a comparative study of acyltransferases from seven plant species. In vitro labeling of acyl ...Substrate selectivity of glycerol-3-phosphate acyltransferase （EC 2. 3. 1. 15） of rice （Oryza sativa L.） was explored in a comparative study of acyltransferases from seven plant species. In vitro labeling of acyl carrier protein （ACP） with ^14C or 3H showed that acyltransferase from chill-sensitive plants, such as rice that uses either oleic （18：1） or palmitic acid （16：0） as acyl donor at comparable rates, displays lower selectivity than the enzyme from chill-resistant plants, such as spinach, which preferentially uses oleic acid （18：1） rather than palmitic acid （16：0） as an acyl donor. This may be a result of the size and character of the substrate-binding pocket of acyltransferase. Homology modeling and protein structure-based sequence alignment of acyltransferases revealed that proteins from either chill-sensitive or chill-tolerant plants shared a highly conserved domain containing the proposed substrate-binding pocket. However, the aligned residues surrounding the substrate-binding pocket are highly heterogeneous and may have an influence mainly on the size of the substrate binding pockets of acyltransferases. The substrate selectivity of acyltransferase of rice can be improved by enlarging the substrate-binding pocket using molecular biological methods.展开更多
Background： Collagen Vl-related myopathies are autosomal dominant and recessive hereditary myopathies, mainly including Ullrich congenital muscular dystrophy （UCMD） and Bethlem myopathy （BM）. Muscle magnetic reso...Background： Collagen Vl-related myopathies are autosomal dominant and recessive hereditary myopathies, mainly including Ullrich congenital muscular dystrophy （UCMD） and Bethlem myopathy （BM）. Muscle magnetic resonance imaging （MRI） has been widely used to diagnosis muscular disorders. The purpose of this study was to evaluate the diagnostic value of thigh muscles MRI for collagen VI-related myopathies. Methods： Eleven patients with collagen VI gene mutation-related myopathies were enrolled in this study. MRI of the thigh muscles was performed in all patients with collagen VI gene mutation-related myopathies and in 361 patients with other neuromuscular disorders （disease controls）. Tl-weighted images were used to assess fatty infiltration of the muscles using a modified Mercuri＇s scale. We assessed the sensitivity and specificity of the MRI features of collagen Vl-related myopathies. The relationship between fatty infiltration of muscles and specific collagen VI gene mutations was also investigated. Results： Eleven patients with collagen VI gene mutation-related rnyopathies included six UCMD patients and five BM patients. There was no significant difference between UCMD and BM patients in the fatty infiltration of each thigh muscle except sartorius （P = 0.033）： theretbre, we combined the UCMD and BM data. Mean fatty infiltration scores were 3.1 and 3.0 in adductor magnus and gluteus maximus, while the scores were 1.3, 1.3, and 1.5 in gracilis, adductor longus, and sartorius, respectively. A “target” sign in rectus femoris （RF） was present in seven cases, and a “sandwich” sign in vastus lateralis （VL） was present in ten cases. The “target” and “sandwich” signs had sensitivities of 63.6% and 90.9% and specificities of 97.3% and 96,9% for the diagnosis of collagen Vl-related myopathies, respectively. Fatty infiltration scores were 2.0-3.0 in seven patients with mutations in the triple-helical domain, and 1.0-1.5 in three of four patients with mutations in the N- o展开更多
AIM: Hepatitis B surface antigen (HBsAg) mutant of hepatitisB virus (HBV) is one of the important factors that result inimmune escape and cause failure of immunization. In thisstudy we reported and characterized a nov...AIM: Hepatitis B surface antigen (HBsAg) mutant of hepatitisB virus (HBV) is one of the important factors that result inimmune escape and cause failure of immunization. In thisstudy we reported and characterized a novel HBV mutantwith A-to-G at nt551 and intended to provide theoreticaldata for prevention of HBV infection in China.METHODS: A methodology comprising polymerase chainreaction (PCR) amplifying, M13 bacteriophage cloning andnucleotide sequencing was used to analyze the sera of thepediatric patient who was hepatitis B (HB) immune failure.Expression plasmids containing the mutant S gene and awild-type (adr) S gene were constructed respectively andthe recombinant HBsAg were expressed in COS-7 cells underthe regulation of SV40 early promoter. The recombinantproteins were investigated for their immunological reactivitywith different monoclonal antibodies (mAb) against 'a'determinant and vaccine-raised human neutralizingantibodies.RESULTS: It was found that there was a new point mutationat nt551 of the HBV (adr) genome from A to G, leading to asubstitution of methionine (Met) to valine (Val) at position133 in the 'a' determinant of HBsAg. Compared to the wild-type HBsAg, the binding activity of the muant HBsAg tomAbs (A6, A11 and S17) and to vaccine-raised human anti-hepatitis B surface antibody (anti-HBs) decreased significantly.CONCLUSION: According to the facts that the patient hasbeen immunized with HB vaccine and that the serum is anti-HBs positive and HBsAg negative, and based on thenucleotide sequence analysis of the mutant HBV S geneand its alteration of antigenicity, the HBV is considered tobe a new vaccine-induced immune escape mutant differentfrom the known ones.展开更多
The experimental research on refining slag systems for ultra-low sulphur steel was carried out in a 10 kg induction furnace.It was proved that sulphur element in molten steel can be removed to less than 5×10^(-6)...The experimental research on refining slag systems for ultra-low sulphur steel was carried out in a 10 kg induction furnace.It was proved that sulphur element in molten steel can be removed to less than 5×10^(-6) by adding CaO-Al_2O_3-SiO_2-MgO-CaF_2 slag on the surface of molten steel and feeding CaO-BaO-CaF2 wire into molten steel.And L_s,which is the coefficient of sulphur between slag and molten steel,that is ω(s)/ω[s],increases by increasing I(I = ωBaO/ωCaO).When I=5/3,L_s can be up to its maximum of 633.The CaSi is effective for deep desulphurization,especially when it is added to the slag of wire feeding.展开更多
文摘Background：One-lung ventilation （OLV） is a common ventilation technology during thoracic surgery that can cause serious clinical problems.We aimed to conduct a meta-analysis to compare oxygenation and intrapulmonary shunt during OLV in adults undergoing thoracic surgery with dexmedetomidine （Dex） versus placebo to assess the influence and safety of using Dex.Methods：Randomized controlled trials comparing lung protection in patients who underwent thoracic surgery with Dex or a placebo were retrieved from PubMed,EMBASE,MEDLINE,Cochrane Library,and China CNKI database.The following information was extracted from the paper：arterial oxygen partial pressure （PaO2）,PaO2/inspired oxygen concentration （PaO2/FiO2,oxygenation index [OI]）,intrapulmonary shunt （calculated as Qs/Qt）,mean arterial pressure （MAP）,heart rate （HR）,tumor necrosis factor-α （TNF-α）,interleukin （IL）-6,superoxide dismutase （SOD）,and malondialdehyde （MDA）.Results：Fourteen randomized controlled trials were included containing a total of 625 patients.Compared with placebo group,Dex significantly increased PaO2/FiO2 （standard mean difference [SMD] =0.98,95% confidence interval [CI] [0.72,1.23],P 〈 0.00001）.Besides,Qs/Qt （SMD=-1.22,95% CI [-2.20,-0.23],P =0.020）,HR （SMD=-0.69,95% CI [-1.20,0.17],P =0.009）,MAP （SMD=-0.44,95% CI [-0.84,0.04],P =0.030）,the concentrations ofTNF-α （SMD =-1.55,95% CI [-2.16,-0.95],P 〈0.001）,and IL-6 （SMD =-1.53,95% CI [-2.37,-0.70],P =0.0003） were decreased in the treated group,when compared to placebo group.No significant difference was found in MDA （SMD =-1.14,95% CI [-3.48,1.20],P =0.340） and SOD （SMD =0.41,95% CI [-0.29,1.10],P =0.250） between the Dex group and the placebo group.Funnel plots did not detect any significant publication bias.Conclusions：Dex may improve OI and reduce intrapulmonary shunt during OLV in adults undergoing thoracic surgery.However,this conclusion might be weakened by the limited number of pooled studies and p
文摘BACKGROUND: Pulmonary complications after orthoto- pic liver transplantation (OLT) include high morbidity and mortality. Experimental data have suggested hepatic ische- mia and reperfusion are induced by pro-inflammatory cyto- kines. The high level of inflammatory cytokines might ad- ditionally influence pulmonary cappillary fluid filtration. The objectives of this study were to measure the concentra- tions of tumor necrotic factor-alpha (TNF-α), interleukin- 6 (IL-6) and interleukin-8 (IL-8) during OLT and to in- vestigate the relationship between these cytokines and post- operative pulmonary complications. METHODS: Twenty-two patients undergoing OLT were divided into two groups according to whether they had postoperative pulmonary complications: group A consis- ting of 8 patients with postoperative pulmonary complica- tions , and group B consisting of 14 patients without post- operative pulmonary complications. Enzyme-linked im- munoassay (ELISA) was used to determine serum TNF-α, IL-6 and IL-8. Blood samples were taken at the beginning of operation (T0 ), clamping and cross-clamping of the in- ferior cava and portal vein (T1, T2 ), 90 minutes and 3 hours after reperfusion (T3 , T4 ) and 24 hours after opera- tion (T5). RESULTS: The level of PaO2/FiO2 in group A was lower than that in group B ( P <0. 05 ). The concentrations of TNF-α, IL-6 and IL-8 in the two groups increased rapidly at T2 , peaked at T3 , decreased rapidly after T3 until 24 hours after operation. The concentrations of TNF-α, IL-6 and IL-8 in group A were higher than those in group B at T2, T3, and T4(P<0.05). CONCLUSION: After un-clamping of the inferior cava and portal vein, the serum concentrations of TNF-α, IL-6 and IL-8 increased may be related to pulmonary injury after he- patic ischemic reperfusion.
基金supported by China Meteorological Administration (CMA) Specific Research on ClimateChange (No. CCSF-10-06)the National Key Scientific Research Program of Global Change (No. 2010CB951001)
文摘By using the observation data from 89 weather stations in Xinjiang during 1961-2010, this paper analyzed the basic climatic elements including temperature, precipitation, wind speed, sunshine duration, water vapor pressure, and dust storm in the entire Xinjiang and the subareas: North Xinjiang, Tianshan Mountains, and South Xinjiang. The results indicate that from 1961 to 2010 the annual and seasonal mean temperatures in the entire Xinjiang show an increasing trend with the increasing rate rising from south to north. The increasing rate of annual mean minimum temperature is over twice more than that of the annual mean maximum temperature, contributing much to the increase in the annual averages. The magnitude of the decrease rate of low-temperature days is larger than the increase rate of high-temperature days. The increase of warm days and warm nights and the decrease of cold days and cold nights further reveal that the temperature increasing in Xinjiang is higher. In addition, annual and seasonal rainfalls have been increasing. South Xinjiang experiences higher increase in rainfall amounts than North Xinjiang and Tianshan Mountains. Annual rainy days, longest consecutive rainy days, the daily maximum precipitation and extreme precipitation events, annual torrential rain days and amount, annual blizzard days and amount, all show an increasing trend, corresponding to the increasing in annual mean water vapor pressure. This result shows that the humidity has increased with temperature increasing in the past 50 years. The decrease in annual mean wind speed and gale days lessen the impact of dust storm, sandstorm, and floating dust events. The increase in annual rainy days is the cause of the decrease in annual sunshine duration, while the increase in spring sunshine duration corresponds with the decrease in dust weather. Therefore, the increase in precipitation indicators, the decrease in gales and dust weather, and the increasing in sunshine duration in spring will be beneficial to crops growth.
基金supported by the Special Scientific Research Funds for Commonweal Section of the Ministry of Agriculture, China (nyhyzx07-3-35)the Natural Science Foundation of Zhejiang Province, China (R3090394)the Key Laboratory of Processing and Quality Control of Tea & Beverage Plants Products, Ministry of Agriculture, China (2010K1004)
文摘The relationship between the amount of tea cream and the chemical components contents in the original green tea infusion was investigated. The results showed that the amounts of tea cream in the green tea infusions obtained from different cultivars and different parts of new shoots were varied. There were many chemical components participating in the formation of green tea cream. However, there were only the contents of caffeine （Y=0.85, P〈0.01） and polyphenols （Y=0.65, P〈0.05） in the original green tea infusion highly correlated with the amount of green tea cream. Stepwise regression analysis of overall chemical components indicated that the contents of caffeine and gallated catechins in the original green tea infusion had a significant effect （P〈0.01） on green tea cream levels. Cream （g L-1）=-172.071＋ 0.129×Ccaffeine＋0.024×Ggallated catechins （R2=0.936）. The amount of green tea cream can be predicted by the contents of gallated catechins and caffeine in the original tea infusion. Principal component analysis also indicated that catechins, minerals, and polysaccharides were the important chemical components in the formation of green tea cream.
文摘BACKGROUND With the increasingly extensive application of artificial intelligence(AI)in medical systems,the accuracy of AI in medical diagnosis in the real world deserves attention and objective evaluation.AIM To investigate the accuracy of AI diagnostic software(Shukun)in assessing ischemic penumbra/core infarction in acute ischemic stroke patients due to large vessel occlusion.METHODS From November 2021 to March 2022,consecutive acute stroke patients with large vessel occlusion who underwent mechanical thrombectomy(MT)post-Shukun AI penumbra assessment were included.Computed tomography angiography(CTA)and perfusion exams were analyzed by AI,reviewed by senior neurointerventional experts.In the case of divergences among the three experts,discussions were held to reach a final conclusion.When the results of AI were inconsistent with the neurointerventional experts’diagnosis,the diagnosis by AI was considered inaccurate.RESULTS A total of 22 patients were included in the study.The vascular recanalization rate was 90.9%,and 63.6%of patients had modified Rankin scale scores of 0-2 at the 3-month follow-up.The computed tomography(CT)perfusion diagnosis by Shukun(AI)was confirmed to be invalid in 3 patients(inaccuracy rate:13.6%).CONCLUSION AI(Shukun)has limits in assessing ischemic penumbra.Integrating clinical and imaging data(CT,CTA,and even magnetic resonance imaging)is crucial for MT decision-making.
基金supported by the National Natural Science Foundation of China(81788101)the National Basic Research Program of China(973 Program 2015CB553800)+3 种基金the National Natural Science Foundation of China(31420103910,81630042,31500737,and 31601122)the China Postdoctoral Science Foundation(2018T110638,2017 M620267,and 2015T80680)the 111 Project(B12001)the National Science Foundation of China for Fostering Talents in Basic Research(J1310027).
文摘Interferons(IFNs)play an important role in immunomodulatory and antiviral functions.IFN-induced necroptosis has been reported in cells deficient in receptor-interacting protein kinase 1(RIPK1),Fas-associated protein with death domain(FADD),or caspase-8,but the mechanism is largely unknown.Here,we report that the DNA-dependent activator of IFN regulatory factors(ZBP1,also known as DAI)is required for both type Ⅰ(β)and type Ⅱ(γ)IFN-induced necroptosis.We show that L929 fibroblast cells became susceptible to IFN-induced necroptosis when RIPK1,FADD,or Caspase-8 was genetically deleted,confirming the antinecroptotic role of these proteins in IFN signaling.We found that the pronecroptotic signal from IFN stimulation depends on new protein synthesis and identified ZBP1,an IFN-stimulated gene(ISG)product,as the de novo synthesized protein that triggers necroptosis in IFN-stimulated cells.The N-terminal domain(ND)of ZBP1 is important for ZBP1–ZBP1 homointeraction,and its RHIM domain in the C-terminal region interacts with RIPK3 to initiate RIPK3-dependent necroptosis.The antinecroptotic function of RIPK1,FADD,and caspase-8 in IFN-treated cells is most likely executed by caspase-8-mediated cleavage of RIPK3,since the inhibitory effect on necroptosis was eliminated when the caspase-8 cleavage site in RIPK3 was mutated.ZBP1-mediated necroptosis in IFN-treated cells is likely physiologically relevant,as ZBP1 KO mice were significantly protected against acute systemic inflammatory response syndrome(SIRS)induced by TNF+IFN-γ.
文摘Background：Dysferlinopathy is caused by mutations in the dysferlin （DYSF） gene.Here,we described the genetic features of a large cohort of Chinese patients with this disease.Methods：Eighty-nine index patients were included in the study.DYSF gene analysis was performed by Sanger sequencing in 41 patients and targeted next generation sequencing （NGS） in 48 patients.Multiplex ligation-dependent probe amplification （MLPA） was performed to detect exon duplication/deletion in patients with only one pathogenic mutation.Results：Among the 89 index patients,79 patients were demonstrated to carry two disease-causing （73 cases） or possibly disease-causing mutations （6 cases）,including 26 patients with homozygous mutations.We identified 105 different mutations,including 59 novel ones.Notably,in 13 patients in whom only one pathogenic mutation was initially found by Sanger sequencing or NGS,3 were further identified to carry exon deletions by MLPA.The mutations identified in this study appeared to cluster in the N-terminal region.Mutation types included missense mutations （30.06%）,nonsense mutations （1 7.18%）,frameshift mutations （30.67%）,in-frame deletions （2.45%）,intronic mutations （17.79%）,and exonic rearrangement （1.84%）.No genotype-phenotype correlation was identified.Conclusions：DYSF mutations in Chinese patients clustered in the N-terminal region of the gene.Exonic rearrangements were found in 23% of patients with only one pathogenic mutation identified by Sanger sequencing or NGS.The novel mutations found in this study greatly expanded the mutational spectrum of dysferlinopathy.
基金the innovation Platform Project of Science and Technology,Fujian Province(2021Y2012)the Key Project on the Integration of Industry,Education and Research Collaborative Innovation of Fujian Province(No.2021YZ034011)+2 种基金the Key Project on Science and Technology Program of Fujian Health Commission(No.2021ZD01002)the Fujian Provincial Health and Young and Middle-aged Key Personnel Training Program(No.2022GGA035)the Natural Science Foundation of Fujian Province(No.2023J011221).
文摘This study aimed to investigate the effects of male hepatitis B virus(HBV)infection on male fertility,embryonic development,and in vitro fertilization/intracytoplasmic sperm injection(IVF/ICSI)outcomes.We performed a retrospective cohort study that included 3965 infertile couples who received fresh embryo transfer cycles for the first time at the Fujian Maternity and Child Health Hospital(Fuzhou,China)from January 2018 to January 2021.Infertile couples were categorized based on their HBV infection status into the HBV group(HBV-positive men and HBV-negative women)and the control group(HBV-negative couples).A 1:1 propensity score matching was performed with relatively balanced covariates.Baseline characteristics,semen parameters,laboratory outcomes,clinical outcomes,and obstetric and neonatal outcomes were compared between groups.After propensity score matching,821 couples were included in each group.Both groups had similar semen parameters and obstetric and neonatal outcomes.The HBV group showed a significantly lower live birth rate than the control group(P<0.05).The HBV group had a significantly higher abortion rate than the control group(P<0.05).The rates of high-quality embryos and blastocyst formation were significantly lower in the HBV group than those in the control group(both P<0.05).In conclusion,in couples who undergo IVF/ICSI,male HBV infection reduces the live birth rate and increases the risk of miscarriage.However,the incidence of low birth weight in women with IVF/ICSI does not increase with male HBV infection.
基金Supported by Wuxi Science and Technology Development Fund,No.WX03-02B0205-072001-10.
文摘BACKGROUND Severe acute pancreatitis(AP)is one of the most common diseases of the gastrointestinal tract and carries a significant financial burden with high disability and mortality.There are no effective drugs in the clinical management of severe AP,and there is an absence of evidence-based medicine concerning the treatment of severe AP.AIM To explore whether ulinastatin(UTI)can improve the outcome of severe AP.METHODS The present research included patients who were hospitalized in intensive critical care units(ICUs)after being diagnosed with severe AP.Patients received UTI(400000 IU)or placebos utilizing computer-based random sequencing(in a 1:1 ratio).The primary outcome measures were 7-d mortality,clinical efficacy,inflammatory response,coagulation function,infection,liver function,renal function,and drug-related adverse effects were evaluated.RESULTS A total of 181 individuals were classified into two groups,namely,the placebo group(n=90)and the UTI group(n=91).There were no statistically significant differences in baseline clinical data between the two groups.The 7-d mortality and clinical efficacy in the UTI group were remarkably improved compared with those in the placebo group.UTI can protect against hyperinflammation and improve coagulation dysfunction,infection,liver function,and renal function.UTI patients had markedly decreased hospital stays and hospitalization expenditures compared with the placebo group.CONCLUSION The findings from the present research indicated that UTI can improve the clinical outcomes of patients with severe AP and has fewer adverse reactions.
基金funded by the National Basic Research Program of China (973 Program,2004CB117500)
文摘Microbiota in the gastrointestinal tract （GIT） of piglets during weaning transition can experience a sharp change which could result in growth reduction and diarrhea of weaned piglets. Dietary manipulations can play an important role in attenuating such changes caused by weaning stress. Therefore, ileal and colonic contents of weaned piglets were used as inocula, mannan oligosaccharide （MOS） or sugar beet pulp （SBP） was supplied as single energy sources to investigate effects of MOS or SBP on the shifts of gastro-intestinal microflora and lactobacilli populations. The universal bacteria- and lactobacilli-specific PCR/denaturing gradient gel electrophoresis （DGGE）, cloning and sequencing techniques were used. DGGE profiles of the universal bacteria showed that great changes were found in the position, numbers and intensity of dominant bands after fermentation. The similarity of bacterial community between ileum and colon was increased to 85-97% by MOS or SBP treatment after fermentation from the similarity with 20% before fermentation. MOS treatment significantly increased the bacterial diversity and band number in both ileal and colonic fermentation （P〈0.05）. SBP treatment significantly increased the bacterial diversity and band number in colon （P〈0.05）. It implies that some species were enriched by the addition of MOS or SBP to increase the similarity and diversity of bacterial community in weaned piglets. Five specific bands appearing in MOS or SBP treatment group after fermentation were cloned and sequenced, the changes of species related to Prevotella and Ruminococcus were observed. Two bands related to uncultured bacterium with 98% similarity were detected by MOS or SBP treatment. However, there were no effects on the similarity, diversity index and lactobacilli species revealed by MOS or SBP treatment. These results imply that MOS or SBP could have beneficial effects on the weaning piglets by stablizing microbiota in the GIT microflora.
基金The Tertiary Education Scientific Research Project of Guangzhou Municipal Education Bureau(No.202235305)is gratefully acnowledged for financial support.
文摘The atom-economical C-F insertion chemistry is emerged as a promising technology for the synthesis of various fluorinated scaffolds,which have wide applications both in the academic and the industrial com-munities.The past three years have witnessed rapid developments in this field.This highlight provides an overview on the evolution according to the fluorinating agents used.
基金the National Natural Science Foundation of China (30270794)Natural Science Foundation of Jiangsu Province (BK2005041, BK2007063)the Natural Science Foundation of Education Bureau of Jiangsu Province(04KJB210107, 06KJB180087).
文摘In an attempt to explore the relationships between phosphatidylglycerol （PG） molecular species of thylakoid membrane lipids and sensitivities to chilling-induced photoinhibition, PG molecular species, D1 protein, electron transport activities of thylakoid membrane and the potential quantum yield （FvlFm） in rice treated under middle and low photon flux density （PFD） at 11℃ were analyzed by high performance liquid chromatography, enzyme hydrolysis, gas phase chromatography （GC） and so on. Results showed that the major molecular species of PGs in rice thylakoid membrane were 18：3/16：0, 18：3/16：1（3t）, 18：2/16：0, 18：2/16：1（3t）, 18：1/16：0, 18：1/16：1（3t）, 16：0/16：0, 16：0/16：1（3t）. There were large differences in the contents of unsaturated PG molecular species such as 18：1-3/16：0-16：1（3t） and saturated PG molecular species like 16：0/16：0-16：1（3t） among japonica cv 9516 0-9516）, japonica-indica hybrid F1 j-9516/i-SY63 （ji-95SY） and indica cv Shanyou 63 （i-SY63）. J-9516 containing higher contents of unsaturated PG molecular species was manifest in stable D1 protein contents under chill and tolerant to chill-induced photoinhibition. In contrast to j-9516, i-SY63 with lower contents of unsaturated PG molecular species, exhibited unstable D1 protein contents under chill and was sensitive to chill-induced photoinhibition, ji-95SY containing middle contents of unsaturated PG molecular species between those of j-9516 and i-SY63, exhibited mid extent of sensitivity to chill-induced photoinhibition. The losses in D1 protein also account for the inhibition in electron transport activity of thylakoid membrane and the observed decline in FvlFm. The PG molecular species that is efficient in raising chilling-resistant capacity were those containing unsaturated fatty acids, namely, unsaturated PG molecular species. These results implied that the substrate selectivity of the glycerol-3-phosphate acyltransferase in chloroplasts towards 16：0
基金Supported by the National Natural Science Foundation of China （30270794） and the Jiangsu Provincial Natural Science Foundation of China （BK2005041）.
文摘Temperature is one of the abiotic factors limiting growth and productivity of plants. In the present work, the effect of low non-freezing temperature, as an inducer of ＂chilling resistance＂, was studied in three cultivars of rice （Oryza sativa L.）, japonica cv. 9516 （j-9516）, the two parental lines of superhigh-yield hybrid rice between subspecies, Peiai/E32 （ji-PE）, and the traditional indica hybrid rice Shanyou 63 （i-SY63）. Leaves of chill-treated rice showed chilling-induced resistance, as an increase of their low-temperature tolerance was measured using chlorophyll fluorescence measurements, revealing a change in photosystem II （PSII） efficiency. After 5 d of exposure to 11 ~C under low light （100 pmol·m^-2·s^-1）, levels of unsaturated fatty acids in PSII thylakoid membrane lipids decreased during the initial 1-2 d, then increased slowly and reached 99.2%, 95.3% and 90.1% of the initial value （0 d） in j-9516, ji-PE and i-SY63, respectively, on the fifth day. However, under medium light （600 μmol·m^-2·s^-1）, all cultivars experienced similar substantial photoinhibition, which approached steady state levels after a decline in levels of unsaturated fatty acids in PSII thylakoid membrane lipids to about 57.1%, 53.8% and 44.5% of the initial values （0 d） in j-9516, ji-PE and i-SY63 on the fifth day. Under either chilling-induced resistance （the former） or low temperature photoinhibition （the latter） conditions, the changes of other physiological parameters such as D1 protein contents, electron transport activities of PSII （ETA）, Fv/Fm, xanthophyl cycle activities expressed by DES （deepoxide state） were consistent with that of levels of unsaturated fatty acids in PSII thylakoid membrane lipids. So there were negative correlations between saturated levels of fatty acids （16：1（3t）, 16：0, 18：0）, especially the 16：1（3t） fatty acid on thylakoid membrane and other physiological parameters, such as D1 protein contents, ETA and （A＋Z）/（
基金This study was supported by the grant from Natural Science Foundation of Guangdong Province, China, 2005 （No. 5001673）.
文摘Background Mutations of the LMNA gene encoding lamin A and C are associated with dilated cardiomyopathy （DCM）, conduction system defects and skeletal muscle dystrophy. Here we report a family with a mutation of the LMNA gene to identify the relationship between genotype and phenotype. Methods All 30 members of the family underwent clinical and genetic evaluation. A mutation analysis of the LMNA gene was performed. All of the 12 exons of LMNA gene were extended with polymerase chain reaction （PCR） and the PCR products were screened for gene mutation by direct sequencing. Results Ten members of the family had limb-girdle muscular dystrophy （LGMD） and 6 are still alive. Two patients suffered from DCM. Cardiac arrhythmias included atrioventricular block and atrial fibrillation; sudden death occurred in 2 patients. The pattern of inheritance was autosomal dominant. Mutation c.73C〉G （R25G） in exon 1 encoding the globular domains was confirmed in all of the affected members, resulting in the conversion of arginine （Arg） to glycine （Gly）. Conclusions The mutation R25G in exon 1 of LMNA gene we reported here in a Chinese family had a phenotype of malignant arrhythmia and mild LGMD, suggesting that patients with familial DCM, conduction system defects and skeletal muscle dystrophy should be screened by genetic testing for the LMNA gene.
基金Supported by the National Natural Science Foundation of China (30270794)the Jiangsu Provincial Science Foundation of China (BK2007063 and BK2005041)the Natural Science Foundation of the Education Bureau of Jiangsu Province (06KJB180087 and 04KJB210107)
文摘Substrate selectivity of glycerol-3-phosphate acyltransferase （EC 2. 3. 1. 15） of rice （Oryza sativa L.） was explored in a comparative study of acyltransferases from seven plant species. In vitro labeling of acyl carrier protein （ACP） with ^14C or 3H showed that acyltransferase from chill-sensitive plants, such as rice that uses either oleic （18：1） or palmitic acid （16：0） as acyl donor at comparable rates, displays lower selectivity than the enzyme from chill-resistant plants, such as spinach, which preferentially uses oleic acid （18：1） rather than palmitic acid （16：0） as an acyl donor. This may be a result of the size and character of the substrate-binding pocket of acyltransferase. Homology modeling and protein structure-based sequence alignment of acyltransferases revealed that proteins from either chill-sensitive or chill-tolerant plants shared a highly conserved domain containing the proposed substrate-binding pocket. However, the aligned residues surrounding the substrate-binding pocket are highly heterogeneous and may have an influence mainly on the size of the substrate binding pockets of acyltransferases. The substrate selectivity of acyltransferase of rice can be improved by enlarging the substrate-binding pocket using molecular biological methods.
文摘Background： Collagen Vl-related myopathies are autosomal dominant and recessive hereditary myopathies, mainly including Ullrich congenital muscular dystrophy （UCMD） and Bethlem myopathy （BM）. Muscle magnetic resonance imaging （MRI） has been widely used to diagnosis muscular disorders. The purpose of this study was to evaluate the diagnostic value of thigh muscles MRI for collagen VI-related myopathies. Methods： Eleven patients with collagen VI gene mutation-related myopathies were enrolled in this study. MRI of the thigh muscles was performed in all patients with collagen VI gene mutation-related myopathies and in 361 patients with other neuromuscular disorders （disease controls）. Tl-weighted images were used to assess fatty infiltration of the muscles using a modified Mercuri＇s scale. We assessed the sensitivity and specificity of the MRI features of collagen Vl-related myopathies. The relationship between fatty infiltration of muscles and specific collagen VI gene mutations was also investigated. Results： Eleven patients with collagen VI gene mutation-related rnyopathies included six UCMD patients and five BM patients. There was no significant difference between UCMD and BM patients in the fatty infiltration of each thigh muscle except sartorius （P = 0.033）： theretbre, we combined the UCMD and BM data. Mean fatty infiltration scores were 3.1 and 3.0 in adductor magnus and gluteus maximus, while the scores were 1.3, 1.3, and 1.5 in gracilis, adductor longus, and sartorius, respectively. A “target” sign in rectus femoris （RF） was present in seven cases, and a “sandwich” sign in vastus lateralis （VL） was present in ten cases. The “target” and “sandwich” signs had sensitivities of 63.6% and 90.9% and specificities of 97.3% and 96,9% for the diagnosis of collagen Vl-related myopathies, respectively. Fatty infiltration scores were 2.0-3.0 in seven patients with mutations in the triple-helical domain, and 1.0-1.5 in three of four patients with mutations in the N- o
文摘AIM: Hepatitis B surface antigen (HBsAg) mutant of hepatitisB virus (HBV) is one of the important factors that result inimmune escape and cause failure of immunization. In thisstudy we reported and characterized a novel HBV mutantwith A-to-G at nt551 and intended to provide theoreticaldata for prevention of HBV infection in China.METHODS: A methodology comprising polymerase chainreaction (PCR) amplifying, M13 bacteriophage cloning andnucleotide sequencing was used to analyze the sera of thepediatric patient who was hepatitis B (HB) immune failure.Expression plasmids containing the mutant S gene and awild-type (adr) S gene were constructed respectively andthe recombinant HBsAg were expressed in COS-7 cells underthe regulation of SV40 early promoter. The recombinantproteins were investigated for their immunological reactivitywith different monoclonal antibodies (mAb) against 'a'determinant and vaccine-raised human neutralizingantibodies.RESULTS: It was found that there was a new point mutationat nt551 of the HBV (adr) genome from A to G, leading to asubstitution of methionine (Met) to valine (Val) at position133 in the 'a' determinant of HBsAg. Compared to the wild-type HBsAg, the binding activity of the muant HBsAg tomAbs (A6, A11 and S17) and to vaccine-raised human anti-hepatitis B surface antibody (anti-HBs) decreased significantly.CONCLUSION: According to the facts that the patient hasbeen immunized with HB vaccine and that the serum is anti-HBs positive and HBsAg negative, and based on thenucleotide sequence analysis of the mutant HBV S geneand its alteration of antigenicity, the HBV is considered tobe a new vaccine-induced immune escape mutant differentfrom the known ones.
基金Item Sponsored by National Key Fundamental Research Development Project of China(G1998061500)
文摘The experimental research on refining slag systems for ultra-low sulphur steel was carried out in a 10 kg induction furnace.It was proved that sulphur element in molten steel can be removed to less than 5×10^(-6) by adding CaO-Al_2O_3-SiO_2-MgO-CaF_2 slag on the surface of molten steel and feeding CaO-BaO-CaF2 wire into molten steel.And L_s,which is the coefficient of sulphur between slag and molten steel,that is ω(s)/ω[s],increases by increasing I(I = ωBaO/ωCaO).When I=5/3,L_s can be up to its maximum of 633.The CaSi is effective for deep desulphurization,especially when it is added to the slag of wire feeding.
