mtDNA 突变导致的 OXPHOS 缺陷：谷氨酰胺来拯救！
OXPHOS Defects Due to mtDNA Mutations: Glutamine to the Rescue!
机构信息
Department of Medical Biochemistry, Semmelweis University, Budapest 1094, Hungary.
出版信息
Cell Metab. 2018 Jun 5;27(6):1165-1167. doi: 10.1016/j.cmet.2018.05.010.
PMID:29874564
Abstract
Mutations in mtDNA associated with OXPHOS defects preclude energy harnessing by OXPHOS. The work of Chen et al. (2018) is previewed, reporting flux pathways of glutamine catabolism in mtDNA mutant cells yielding high-energy phosphates through substrate-level phosphorylation and the influence exerted by the severity of OXPHOS impairment.
摘要
与 OXPHOS 缺陷相关的 mtDNA 突变排除了 OXPHOS 通过能量收集。Chen 等人的工作（2018）被预览，报告了 mtDNA 突变细胞中谷氨酰胺分解代谢的通量途径，通过底物水平磷酸化产生高能磷酸化合物，以及 OXPHOS 损伤严重程度的影响。
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