| In CNS,oligodendrocytes generates myelin sheath and wraps axons of neurons,which can maintain the function of of nerve signals of CNS.If its development and the formation of myelin sheaths are abnormal,it will affect the normal transmission of neural signals and damage the cognitive or motor functions of the brain,eventually causing myelin-related neurological diseases such as white matter malnutrition and MS.COPII complex is a polycomplex formed by the participation of Sarlp,Sec23p/Sec24p and Sec13p/Sec31p.It is a ubiquitous intracellular structure in eukaryotic cells and mediates the ER-Golgi transport process of proteins and other substances.COP? complex is widely found in oligodendrocytes and other cells with active synthetic transport secretory activities,but its role in oligodendrocyte line development is not very clear.We experimentally found that Sec 13,a component of the COP? complex,is selectively up-regulated during the development of OLs.Meanwhile,in the OLs lines of mouse models,conditional knockout of Sec 13 would lead to significant myelin formation defects in the CNS of mice,accompanied by tremor and motor coordination and other symptoms,and eventually individual death occurred around the 14th day after birth.Further research found that Sec 13 is also essential for the development of isolated OPCs to form OLs and the development of myelin in the early postpartum period,suggesting that this is a cell autonomous physiological behavior.Tracing back to the source,the study found that Sec 13 was necessary for the normal structure and function of COPII complex in OPCs,and its absence led to endoplasmic reticulum transport obstacles and a large accumulation of substances,leading to its abnormal volume expansion and ER stress and UPR.At the same time,blocking ER-Golgi transport route by BFA has similar results to Sec13cKO.Finally,the study revealed that such a long time and high intensity ER stress finally led to the fate of oligodendrocytes into apoptosis. |
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