OBJECTIVE: @entity53861 ( @entity53861 ) is an essential factor for the initiation of protein synthesis. Mutations in @entity53861 encoded by @entity53861 -5 cause a lethal @entity62 ( @entity62 ). Previous studies have suggested that an improper activated unfolded protein response (UPR) after @entity1642 ( @entity1642 ) contributed to the pathogenesis of the disease. Autophagy, an important compensatory pathway after @entity1642 , was analyzed in this study. METHODS: To determine the tolerance differences to @entity1642 , cell viability and apoptosis rates were detected in oligodendrocyte cell lines transfected with @entity38132 - @entity69022 or the wild type. Autophagy flux was measured between groups. Autophagy inducers and inhibitors were used to identify the role of autophagy in the mutant oligodendrocytes. RESULTS: We confirmed that oligodendrocytes with mutant @entity38132 was less tolerant to @entity1642 than the wild type, with decreased cell viability and increased apoptosis rates. Autophagy flux was depressed in mutant oligodendrocytes under baseline condition and after @entity1642 stimulation. Reduced expression of @entity29332 and @entity10615 were involved in the @entity308 of autophagy flux. The mutant oligodendrocytes pretreated with autophagy inducers showed stable cell viability and decreased apoptosis despite @entity1642 induction, whereas the autophagy inhibitors aggravated cell apoptosis and @entity810 . CONCLUSIONS: Oligodendrocytes transfected with mutant @entity38132 was less tolerant to @entity1642 than the wild type. Depressed autophagy flux was observed in the mutant cells at baseline and after @entity1642 stimulation. Improperly depressed autophagy played a role in the susceptibility to @entity1642 in @entity38132 mutant oligodendrocytes.

Endoplasmic reticulum stress intolerance in XXXX mutant oligodendrocytes is modulated by depressed autophagy.

@entity29332 :: ('64422', 'Gene') :: ['autophagy related gene (Atg) 3'] @entity308 :: ('MESH:D003866', 'Disease') :: ['depression'] @entity38132 :: ('8891', 'Gene') :: ['EIF2B3'] @entity53861 :: ('1967', 'Gene') :: ['Eukaryotic translation initiation factor 2B', 'EIF2B1', 'eIF2B'] @entity69022 :: ('c|SUB|T|1037|C', 'DNAMutation') :: ['c.1037T>C'] @entity10615 :: ('10533', 'Gene') :: ['Atg 7'] @entity810 :: ('MESH:D058186', 'Disease') :: ['viability declination'] @entity62 :: ('MESH:D056784', 'Disease') :: ['VWM', 'leukoencephalopathy-vanishing white matter disease'] @entity1642 :: ('MESH:D015775', 'Disease') :: ['endoplasmic reticulum stress', 'ERS']
@entity38132 :: (8891,Gene) :: ['EIF2B3']
