有哪些信誉好的足球投注网站aberrant localization of fus and tdp43 is associated with misfolding of sod1 in amyotrophic lateral sclerosis异常的本地化付和tdp43与错误折叠sod1的肌萎缩性脊髓侧索硬化症.pdfVIP
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aberrant localization of fus and tdp43 is associated with misfolding of sod1 in amyotrophic lateral sclerosis异常的本地化付和tdp43与错误折叠sod1的肌萎缩性脊髓侧索硬化症
Aberrant Localization of FUS and TDP43 Is Associated
with Misfolding of SOD1 in Amyotrophic Lateral Sclerosis
1 1 1 1 2
Edward Pokrishevsky , Leslie I. Grad , Masoud Yousefi , Jing Wang , Ian R. Mackenzie ,
Neil R. Cashman1*
1 Brain Research Centre, University of British Columbia, Vancouver, Canada, 2 Department of Pathology and Laboratory Medicine, University of British Columbia,
Vancouver, Canada
Abstract
Background: Amyotrophic lateral sclerosis (ALS) is incurable and characterized by progressive paralysis of the muscles of
the limbs, speech and swallowing, and respiration due to the progressive degeneration of voluntary motor neurons.
Clinically indistinguishable ALS can be caused by genetic mutations of Cu/Zn superoxide dismutase (SOD1), TAR-DNA
binding protein 43 (TDP43), or fused in sarcoma/translocated in liposarcoma (FUS/TLS), or can occur in the absence of
known mutation as sporadic disease. In this study, we tested the hypothesis that FUS/TLS and TDP43 gain new pathogenic
functions upon aberrant accumulation in the cytosol that directly or indirectly include misfolding of SOD1.
Methodology/Principal Findings: Patient spinal cord necropsy immunohistochemistry with SOD1 misfolding-specific
antibodies revealed misfolded SOD1 in perikarya and motor axons of SOD1-familial ALS (SOD1-FALS), and in motor axons of
R521C-FUS FALS and sporadic ALS (SALS) with cytoplasmic TDP43 inclusions. SOD1 misfolding and oxidation was also
detected using immunocytochemistry and quantitative immunoprecipitation of human neuroblastoma SH-SY5Y cells as
well as cultured murine spinal neural cells transgenic for human wtSOD1, which were transientl
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