title: TDP-43 in skeletal muscle of patients affected with amyotrophic lateral sclerosis.
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.
authors.affiliation: Department of Neurological Sciences, University of Padova, Italy. gianni.soraru@unipd.it <gianni.soraru@unipd.it>
authors.forename: Gianni
authors.initials: G
authors.lastname: Sorarú
authors.forename: Valeria
authors.initials: V
authors.lastname: Orsetti
authors.forename: Emanuele
authors.initials: E
authors.lastname: Buratti
authors.forename: Francisco
authors.initials: F
authors.lastname: Baralle
authors.forename: Valentina
authors.initials: V
authors.lastname: Cima
authors.forename: Marco
authors.initials: M
authors.lastname: Volpe
authors.forename: Carla
authors.initials: C
authors.lastname: D'ascenzo
authors.forename: Arianna
authors.initials: A
authors.lastname: Palmieri
authors.forename: Kostantinos
authors.initials: K
authors.lastname: Koutsikos
authors.forename: Elena
authors.initials: E
authors.lastname: Pegoraro
authors.forename: Corrado
authors.initials: C
authors.lastname: Angelini
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000378:metabolism* / Q000473:pathology*; D001706:Biopsy; D015153:Blotting, Western; D001921:Brain / Q000378:metabolism / Q000473:pathology; D004268:DNA-Binding Proteins / Q000378:metabolism*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000378:metabolism* / Q000473:pathology*
chemical_list: D004268:DNA-Binding Proteins