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Histopathological Diagnostic Marker for ALS: Phosphorylated Transacting Response DNA-Binding Protein of 43kDa in Intramuscular Nerve Bundles Takashi Kurashige 1 1Department of Neurology, National Hospital Organization Kure Medical Center and Chugoku Cancer Center Keyword: 筋萎縮性側索硬化症 , 筋内神経束 , TDP-43 , 筋生検 , 組織診断マーカー , amyotrophic lateral sclerosis , intramuscular nerve bundle , muscle biopsy , histopathological diagnostic marker pp.877-887
Published Date 2023/7/1
DOI https://doi.org/10.11477/mf.1416202436
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Abstract

The discovery of transacting response DNA-binding protein of 43 kDa (TDP-43) led to a deeper understanding of the pathogenesis of amyotrophic lateral sclerosis (ALS). Since this discovery, blood and cerebrospinal fluid biomarkers of ALS have been reported. However, these biomarkers do not exhibit sufficient specificity for ALS. Our case-control postmortem and retrospective muscle biopsy cohort studies revealed phosphorylated TDP-43 in intramuscular nerve bundles, which precedes the clinical fulfillment of the Gold Coast criteria. We attempted to establish a histopathological biomarker for ALS and identify molecular targets for the treatment of lower motor dysfunction in patients with ALS.


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電子版ISSN 1344-8129 印刷版ISSN 1881-6096 医学書院

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