Ubiquitin homeostasis disruption, a common cause of proteostasis collapse in amyotrophic lateral sclerosis?

作     者：Christen G.Chisholm Jeremy S.Lum Natalie E.Farrawell Justin J.Yerbury Christen G.Chisholm;Jeremy S.Lum;Natalie E.Farrawell;Justin J.Yerbury

作者机构：Illawarra Health and Medical Research Institute/Molecular Horizons and School of Chemistry and Molecular BioscienceScience Medicine and Health FacultyUniversity of WollongongNorthfields AveWollongongNSWAustralia 

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

年 卷 期：2022年第17卷第10期

页      面：2218-2220页

核心收录：

学科分类：1002[医学-临床医学] 100204[医学-神经病学] 10[医学] 

基　　金：supported by an NHMRC Investigator Grant(No.1194872,to JJY) a NHMRC Dementia Teams Grant(No.1095215 both to JJY and NEF) a UOW-Yerbury Family Scholarship(to CGC) a Fight MND Drug Development Grant(to JJY) 

主　　题：stasis homeostasis collapse 

摘      要：Amyotrophic lateral sclerosis(ALS)is associated with proteostasis collapse:ALS is an unrelenting neurodegenerative disease that is characterized by the loss of motor neurons in the brain and spinal cord,resulting in the progressive atrophy,and eventual paralysis,of skeletal *** due to respiratory failure usually occurs within 2-5 years from symptom *** 90%of ALS cases are of unknown etiology and are termed sporadic ALS(sALS).The remaining 10%of ALS cases present with a family history(familial ALS;fALS)and are associated with genetic mutations in a range of over 20 functionally heterogeneous *** of disease origin,the pathological hallmark of ALS is the accumulation of ubiquitylated protein inclusions in motor neurons and surrounding glial *** presence of these inclusions,compromised largely of misfolded and aggregated proteins,implies a collapse in *** refers to the maintenance of the proteome in a state of balance or equilibrium so that a cell can perform its proper function.