Tale of mitochondria and mitochondria-associated ER membrane in patient-derived neuronal models of Wolfram syndrome  

作　　者：Laetitia Aubry Timothy Barrett Sovan Sarkar 

机构地区：[1]I-STEM,CECS,Corbeil-Essonnes,France [2]UniversitéParis-Saclay,Universitéd’Evry,INSERM,I-STEM,UMR861,Corbeil-Essonnes,France [3]Institute of Cancer and Genomic Sciences,Institute of Biomedical Research,College of Medical and Dental Sciences,University of Birmingham,Edgbaston,Birmingham,UK [4]Department of Endocrinology,Birmingham Women’s and Children’s Hospital,Steelhouse Lane,Birmingham,UK

出　　处：《Neural Regeneration Research》2025年第9期2587-2588,共2页中国神经再生研究（英文版）

基　　金：supported by LifeArc Philanthropic Fund(P2019-0004);LifeArc Pathfinder Award;along with Wellcome Trust Seed Award(109626/Z/15/Z);FA PESP-UoB Strategic Collaboration Fund;Birmingham Fellowship(to SS);grants from Laboratoire d'Excellence Revive(Investissement d'Avenir;ANR-10-LABX-73);the Region lle-de-France via doctoral school Innovation Therapeutique,du Fondamentalàl'Appliqué(ED569)from Universite Paris-Saclay(to LA);Medical Research Council(MRC)Developmental Pathway Funding Scheme(DPFS)grant(MR/P007732/1)(to TB);supported by the Association Fran?aise contre les Myopathies(AFM-Téléthon)。

摘　　要：Mitochondria and mitochondria-associated endoplasmic reticulum membrane in neurodegenerative diseases:Mitochondria generate most of the chemical energy needed to power the biochemical reactions of cells,and thus are often referred to as the"powerhouse"of the cell.Nevertheless,this organelle is also involved in a pleth,ora of different cellular functions such as calcium(Ca^(2+))homeostasis,apoptosis,oxidative stress,and several metabolic pathways including oxidative phosphorylation,tricarboxylic acid cycle,andβ-oxidation of fatty acids.

关 键 词：OXIDATIVE CYCLE carboxylic 

分 类 号：R741[医药卫生—神经病学与精神病学]