TDP-43 in skeletal muscle of patients affected with amyotrophic lateral sclerosis

Gianni Sorarú, Valeria Orsetti, Emanuele Buratti, Francisco Baralle, Valentina Cima, Marco Volpe, Carla D'Ascenzo, Arianna Palmieri, Kostantinos Koutsikos, Elena Pegoraro, Corrado Angelini

Research output: Contribution to journalArticlepeer-review

Abstract

TAR DNA binding protein (TDP-43) is the pathologic substrate of neuronal and glial aggregates in amyotrophic lateral sclerosis (ALS). Pathologic TDP-43 is hyperphosphorylated and cleaved to generate abnormal protein species that accumulate in the cytoplasm. To assess the hypothesis of TDP-43 pathology as a systemic disorder in ALS we analysed the immunohistochemical and biochemical profile of TDP-43 in muscle biopsies of 30 ALS patients and 30 controls. In all ALS muscle biopsies we observed that TDP-43 was constantly present in an intranuclear localization and TDP-43 Western blotting showed only a 43-KDa band as controls. Our results suggest that TDP-43 pathology is probably confined to the central nervous system in ALS.

Original languageEnglish
Pages (from-to)240-243
Number of pages4
JournalAmyotrophic Lateral Sclerosis
Volume11
Issue number1-2
DOIs
Publication statusPublished - 2010

Keywords

  • Amyotrophic lateral sclerosis
  • Skeletal muscle
  • TDP-43

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

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