典型文献
Ubiquitin homeostasis disruption, a common cause of proteostasis collapse in amyotrophic lateralsclerosis?
文献摘要:
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 muscles. Death due to respiratory failure usually occurs within 2–5 years from symptom onset. Approximately 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 genes. Regardless of disease origin, the pathological hallmark of ALS is the accumulation of ubiquitylated protein inclusions in motor neurons and surrounding glial cells.
文献关键词:
中图分类号:
作者姓名:
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 Bioscience,Science Medicine and Health Faculty,University of Wollongong,Northfields Ave,Wollongong,NSW,Australia*Correspondence to:Justin J.Yerbury,PhD
文献出处:
引用格式:
[1]Christen G.Chisholm;Jeremy S.Lum;Natalie E.Farrawell;Justin J.Yerbury-.Ubiquitin homeostasis disruption, a common cause of proteostasis collapse in amyotrophic lateralsclerosis?)[J].中国神经再生研究(英文版),2022(10):2218-2220
A类:
proteostasis,lateralsclerosis,unrelenting,sALS,fALS,ubiquitylated
B类:
Ubiquitin,homeostasis,disruption,common,cause,collapse,amyotrophic,Amyotrophic,associated,neurodegenerative,disease,that,characterized,by,loss,motor,neurons,brain,spinal,cord,resulting,progressive,atrophy,eventual,paralysis,skeletal,muscles,Death,due,respiratory,failure,usually,occurs,within,years,from,symptom,onset,Approximately,cases,are,unknown,etiology,termed,sporadic,remaining,present,family,history,familial,genetic,mutations,range,over,functionally,heterogeneous,genes,Regardless,origin,pathological,hallmark,accumulation,protein,inclusions,surrounding,glial,cells
AB值:
0.600373
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