Axonal degeneration is one of the key features of neu-rodegenerative disorders.In the canonical view,axonal degeneration destructs neural connections and promotes detrimental disease defects.Here,we assessed the enter...Axonal degeneration is one of the key features of neu-rodegenerative disorders.In the canonical view,axonal degeneration destructs neural connections and promotes detrimental disease defects.Here,we assessed the enteric nervous system(ENS)of the mouse,nonhuman primate,and human by advanced 3D imaging.We observed the profound neurodegeneration of catecholaminergic axons in human colons with ulcerative colitis,and similarly,in mouse colons during acute dextran sulfate sodium-induced colitis.However,we unexpectedly revealed that blockage of such axonal degeneration by the Sarml deletion in mice exacerbated the colitis condition.In contrast,pharmacologic ablation or chemogenetic inhibition of catecholaminergic axons suppressed the colon inflammation.We further showed that the catecholaminergic neurotransmitter norepinephrine exerted a pro-inflammatory function by enhancing the expression of IL-17 cytokines.Together,this study demonstrated that Sarm1-mediated neurodegeneration within the ENS mitigated local inflammation of the colon,uncovering a previously-unrecognized beneficial role of axonal degeneration in this disease context.展开更多
Sudden cardiac death (SCD) in children and adolescents is uncommon and yet it is devastating for both victim's family and the society. Recently, it was increasingly recognized that SCD in young patients with struct...Sudden cardiac death (SCD) in children and adolescents is uncommon and yet it is devastating for both victim's family and the society. Recently, it was increasingly recognized that SCD in young patients with structurally normal heart may be caused by inheritable primary electrical diseases due to the malfunction of cardiac ion channels, a disease entity known as the ion channelopathies. Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a specific form of ion channelopathy which can cause cardiac syncope or SCD in young patients by producing catecholamine-induced bi-directional ventricular tachycardia (BiVT), polymorphic VT and ventricular fibrillation (VF) during physical exertion or emotion. We reported here an index case of CPVT caused by cardiac ryanodine receptor gene (hRyR2) mutation which presented as cardiac syncope and sudden cardiac arrest in a Chinese adolescent female.展开更多
Thermomineral water from the Atomic Spa Gornja Trepca has been used for a century in the treatment of neurologic disease. The thermomineral water contains microelements, including lithium and magnesium, which show neu...Thermomineral water from the Atomic Spa Gornja Trepca has been used for a century in the treatment of neurologic disease. The thermomineral water contains microelements, including lithium and magnesium, which show neural regeneration-promoting effects after central nervous system injury. In this study, we investigated the effects of oral intake of thermomineral water from the Atomic Spa Gornja Trepca on nerve regeneration in a 3-month-old mouse model of spinal cord injury. The mice receiving oral intake of thermomineral water showed better locomotor recovery than those without administration of thermomineral water at 8 and 12 weeks after lower thoracic spinal cord compression. At 12 weeks after injury, sprouting of catecholaminergic axons was better in mice that drank thermomineral water than in those without administration of thermomineral water, but there was no difference in glial reaction to injury between mice with and without administration of thermomineral water. These findings suggest that thermomineral water can promote the nerve regeneration but cannot reduce glial scar formation in a mouse model of spinal cord injury.展开更多
基金This work was funded by the National Key Research and Development Program of China(2019YFA0802003)the National Natural Science Foundation of China(to J.Y.,#31771111,#31970974 and#32061143007)Supports were also provided by Center for Life Sciences,School of Life Sciences,IDG/McGovem Institute for Brain Research,and State Key Laboratory of Membrane Biology at Peking University,by Chinese Institute for Brain Research,and by Institute of Molecular Physiology at The Shenzhen Bay Laboratory.
文摘Axonal degeneration is one of the key features of neu-rodegenerative disorders.In the canonical view,axonal degeneration destructs neural connections and promotes detrimental disease defects.Here,we assessed the enteric nervous system(ENS)of the mouse,nonhuman primate,and human by advanced 3D imaging.We observed the profound neurodegeneration of catecholaminergic axons in human colons with ulcerative colitis,and similarly,in mouse colons during acute dextran sulfate sodium-induced colitis.However,we unexpectedly revealed that blockage of such axonal degeneration by the Sarml deletion in mice exacerbated the colitis condition.In contrast,pharmacologic ablation or chemogenetic inhibition of catecholaminergic axons suppressed the colon inflammation.We further showed that the catecholaminergic neurotransmitter norepinephrine exerted a pro-inflammatory function by enhancing the expression of IL-17 cytokines.Together,this study demonstrated that Sarm1-mediated neurodegeneration within the ENS mitigated local inflammation of the colon,uncovering a previously-unrecognized beneficial role of axonal degeneration in this disease context.
文摘Sudden cardiac death (SCD) in children and adolescents is uncommon and yet it is devastating for both victim's family and the society. Recently, it was increasingly recognized that SCD in young patients with structurally normal heart may be caused by inheritable primary electrical diseases due to the malfunction of cardiac ion channels, a disease entity known as the ion channelopathies. Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a specific form of ion channelopathy which can cause cardiac syncope or SCD in young patients by producing catecholamine-induced bi-directional ventricular tachycardia (BiVT), polymorphic VT and ventricular fibrillation (VF) during physical exertion or emotion. We reported here an index case of CPVT caused by cardiac ryanodine receptor gene (hRyR2) mutation which presented as cardiac syncope and sudden cardiac arrest in a Chinese adolescent female.
基金supported by the Southeast Europe Cooperation, Hamburg, Germany
文摘Thermomineral water from the Atomic Spa Gornja Trepca has been used for a century in the treatment of neurologic disease. The thermomineral water contains microelements, including lithium and magnesium, which show neural regeneration-promoting effects after central nervous system injury. In this study, we investigated the effects of oral intake of thermomineral water from the Atomic Spa Gornja Trepca on nerve regeneration in a 3-month-old mouse model of spinal cord injury. The mice receiving oral intake of thermomineral water showed better locomotor recovery than those without administration of thermomineral water at 8 and 12 weeks after lower thoracic spinal cord compression. At 12 weeks after injury, sprouting of catecholaminergic axons was better in mice that drank thermomineral water than in those without administration of thermomineral water, but there was no difference in glial reaction to injury between mice with and without administration of thermomineral water. These findings suggest that thermomineral water can promote the nerve regeneration but cannot reduce glial scar formation in a mouse model of spinal cord injury.