Loss of homeostatic functions in microglia from a murine model of Friedreich's ataxia  

作　　者：Ilaria Della Valle Martina Milani Simona Rossi Riccardo Turchi Flavia Tortolici Valentina Nesci Alberto Ferri Cristiana Valle Daniele Lettieri-Bar-bato Katia Aquilano Mauro Cozzolino Savina Apolloni Nadia D'Ambrosi 

机构地区：[1]Department of Biology,University of Rome Tor Vergata,Rome 00133,Italy [2]PhD Program in Cellular and Molecular Biology,University of Rome Tor Vergata,Rome 00133,Italy [3]Institute of Translational Pharmacology,National Research Council(CNR),Rome 00133,Italy [4]Santa Lucia Foundation IRCCS,Rome 00179,Italy [5]Department of Systems Medicine,"Tor Vergata"University of Rome,Rome 00133,Italy

出　　处：《Genes & Diseases》2024年第6期60-64,共5页基因与疾病（英文）

基　　金：funded by the National Ataxia Foundation (NAF)and Friedreich's Ataxia Research Alliance (FARA) (n°821396[RG])to Nadia D'Ambrosi;by Next Generation EU PRIN PNRR 2022 (N°P2022JKTWH)to Nadia D'Ambrosi and Mauro Cozzolino,by FARA Research Grant 2019 to Katia Aquilano;by FARA Research Grant 2021 to Daniele Lettieri-Barbato.

摘　　要：Friedreich's ataxia(FRDA)is a rare genetic disorder characterized by motor discoordination and cerebellar involvement due to mutations in the frataxin(FXN)gene,which encodes a mitochondrial protein involved in ironsulfur cluster biogenesis and iron handling."While progress has been made in understanding FRDA's pathophysiology and cerebellar degeneration caused by frataxin deficiency,the role of central nervous system(CNS)-resident nonneuronal cells,as microglia,necessitates further investigation.

关 键 词：INVOLVEMENT DEGENERATION Friedreich 

分 类 号：R74[医药卫生—神经病学与精神病学]