Objective To explore the effect of TGF-a1 on the mRNA and prot ei n expression of connective tissue growth factor (CTGF) in human peritoneal mesot helial cells (HPMCs) and the probable mechanism. Methods Greater oment...Objective To explore the effect of TGF-a1 on the mRNA and prot ei n expression of connective tissue growth factor (CTGF) in human peritoneal mesot helial cells (HPMCs) and the probable mechanism. Methods Greater omenta from hea lthy adults were used for primary culture of HPMCs. The third generation cells w ere stimulated by 5 ng/ml TGF-a1. Immunohistochemistry, Western blot, ELISA an d RT-PCR were employed to examine the follows:the mRNA and protein expression o f CTGF, the mRNA and protein expression of fibronectin (FN) and collagen type Ⅰ (ColⅠ), the protein expression of p-Smad2/3 and its migration in HPMCs. Result s (1)The mRNA expression of CTGF in the treatment groups increased obviously a s compared to the control group, and peaked at 48 h. (2)The protein expression of intracellular CTGF in the control group was very little, whereas it increase d markedly 24 h after TGF-a1 stimulation, and peaked at 48 h. The mRNA express ion of intracellular FN, and the protein expression of supernatent FN, as well a s the mRNA and protein expression of intracellular ColⅠin the treatment group u p-regulated significantly in a time dependent manner as compared to those in th e control group. (3)p-Smad2/3 in HPMCs hardly expressed in the control group( 3%p-Smad2/3-positive cells), and remarkably expressed 15 min after TGF-a1 s timulation(29%), peaked at 1 h (84%) and then decreased after 2 h(37%). Meanw hile, p-Smad2/3 mainly distributed in cytoplasm at 15 min, concentrated in cell nucleus and peri-nucleus at 1 h, and distributed in cytoplasm again at 2 h. Co nclusions TGF-a1 can induce the mRNA and protein expression of CTGF in the pro gression of human peritoneal fibrosis. The probably mechanism is that signaling through Smad pathway in HPMCs can be activated by TGF-a1 stimulation.展开更多
目的研究经基因诊断确诊的遗传性压迫易感性神经病(hereditary neuropathy with liability to pressure palsies,HNPP)患者的临床特点和电生理特征。方法对来自4个家系的5例HNPP患者进行基因诊断,并总结患者的临床特点,同时分析其电生...目的研究经基因诊断确诊的遗传性压迫易感性神经病(hereditary neuropathy with liability to pressure palsies,HNPP)患者的临床特点和电生理特征。方法对来自4个家系的5例HNPP患者进行基因诊断,并总结患者的临床特点,同时分析其电生理特征,包括肌电图(EMG)、运动神经传导速度(MCV)和感觉神经传导速度(SCV)。结果5例患者均存在周围髓鞘蛋白22 (peripheral myelination protein 22,PMP22)基因缺失。HNPP临床主要表现为反复发作的肢体麻木、无力,神经传导存在广泛异常。结论电生理检查对HNPP的诊断很重要,基因检测发现PMP22基因缺失是诊断HNPP的金标准。展开更多
基金supported by Projects in the National Science and Technology Pillar Program in the Eleventh Five-year Plan Period(2006BAI05A07)National High-Tech Research and Development Program of China(863 Program)(2006AA02A408)+5 种基金Major State Basic Research Development Program of China(973 Program)(2006cb500700)National Key Technologies Research and Development Program of China(2004BA720A03)National Natural Science Foundation of China(30400262,30470619,30710303061,30871354,30971585)New Century Excellent Talents in University of Department of EducationKey Project of Natural Science Foundation of Hunan Province(08JJ3048)Graduate Degree Thesis Innovation Foundation of Central South University(2008yb030)
文摘Objective To explore the effect of TGF-a1 on the mRNA and prot ei n expression of connective tissue growth factor (CTGF) in human peritoneal mesot helial cells (HPMCs) and the probable mechanism. Methods Greater omenta from hea lthy adults were used for primary culture of HPMCs. The third generation cells w ere stimulated by 5 ng/ml TGF-a1. Immunohistochemistry, Western blot, ELISA an d RT-PCR were employed to examine the follows:the mRNA and protein expression o f CTGF, the mRNA and protein expression of fibronectin (FN) and collagen type Ⅰ (ColⅠ), the protein expression of p-Smad2/3 and its migration in HPMCs. Result s (1)The mRNA expression of CTGF in the treatment groups increased obviously a s compared to the control group, and peaked at 48 h. (2)The protein expression of intracellular CTGF in the control group was very little, whereas it increase d markedly 24 h after TGF-a1 stimulation, and peaked at 48 h. The mRNA express ion of intracellular FN, and the protein expression of supernatent FN, as well a s the mRNA and protein expression of intracellular ColⅠin the treatment group u p-regulated significantly in a time dependent manner as compared to those in th e control group. (3)p-Smad2/3 in HPMCs hardly expressed in the control group( 3%p-Smad2/3-positive cells), and remarkably expressed 15 min after TGF-a1 s timulation(29%), peaked at 1 h (84%) and then decreased after 2 h(37%). Meanw hile, p-Smad2/3 mainly distributed in cytoplasm at 15 min, concentrated in cell nucleus and peri-nucleus at 1 h, and distributed in cytoplasm again at 2 h. Co nclusions TGF-a1 can induce the mRNA and protein expression of CTGF in the pro gression of human peritoneal fibrosis. The probably mechanism is that signaling through Smad pathway in HPMCs can be activated by TGF-a1 stimulation.
文摘目的研究经基因诊断确诊的遗传性压迫易感性神经病(hereditary neuropathy with liability to pressure palsies,HNPP)患者的临床特点和电生理特征。方法对来自4个家系的5例HNPP患者进行基因诊断,并总结患者的临床特点,同时分析其电生理特征,包括肌电图(EMG)、运动神经传导速度(MCV)和感觉神经传导速度(SCV)。结果5例患者均存在周围髓鞘蛋白22 (peripheral myelination protein 22,PMP22)基因缺失。HNPP临床主要表现为反复发作的肢体麻木、无力,神经传导存在广泛异常。结论电生理检查对HNPP的诊断很重要,基因检测发现PMP22基因缺失是诊断HNPP的金标准。
