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

作     者：Laetitia Aubry Timothy Barrett Sovan Sarkar 

作者机构：I-STEMCECSCorbeil-EssonnesFrance UniversitéParis-SaclayUniversitéd’EvryINSERMI-STEMUMR861Corbeil-EssonnesFrance Institute of Cancer and Genomic SciencesInstitute of Biomedical ResearchCollege of Medical and Dental SciencesUniversity of BirminghamEdgbastonBirminghamUK(Barrett T Department of EndocrinologyBirmingham Women’s and Children’s HospitalSteelhouse LaneBirminghamUK 

出 版 物：《Neural Regeneration Research》 (中国神经再生研究（英文版）)

年 卷 期：2025年第20卷第9期

页      面：2587-2588页

核心收录：

学科分类：081704[工学-应用化学] 07[理学] 08[工学] 0817[工学-化学工程与技术] 070303[理学-有机化学] 0703[理学-化学] 

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) 

主　　题：oxidative cycle carboxylic 

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 thepowerhouseof the ***,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.