Strategies to promote the maturation of ALS-associated SOD1 mutants: small molecules return to the fold

Luke McAlary; Justin J.Yerbury

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Strategies to promote the maturation of ALS-associated SOD1 mutants: small molecules return to the fold

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(Cu)^IIATSM(CuATSM)promotes the proper folding of familial amyotrophic lateral sclerosis(fALS)-associated copper,zinc superoxide dismutase(SOD1):ALS is a progressive neurodegenerative disease that affects motor neurons in the cortex and spinal cord,resulting in paralysis and ultimately death.The majority of cases are sporadic and the remainder are fALS where a subset of cases are associated with over 160,mostly missense,mutations in the SOD1 enzyme structure.Generally,these mutations impede the correct folding of SOD1 by disrupting either metal binding,disulfide formation,and/or dimerization,leading to the accumulation of misfolded SOD1 which can form the intracellular inclusions observed in patient tissue.

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《中国神经再生研究：英文版》 |2019年第9期|1511-1512|共2页
作者
Luke McAlary; Justin J.Yerbury;
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作者单位

Illawarra Health and Medical Research Institute;

Wollongong;

NSW;

Australia;

School of Chemistry and Molecular Bioscience;

Molecular Horizons;

Faculty of Science;

Medicine and Health;

University of Wollongong;

NSW;

Australia;

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原文格式 PDF
正文语种 chi
中图分类医药、卫生;
关键词
(Cu)^IIATSM(CuATSM); familial; amyotrophic; patient; tissue;

Strategies to promote the maturation of ALS-associated SOD1 mutants: small molecules return to the fold

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