TDP-43 in skeletal muscle of patients affected with amyotrophic lateral sclerosis.
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.
Sorarú
Gianni
G
Department of Neurological Sciences, University of Padova, Italy. gianni.soraru@unipd.it <gianni.soraru@unipd.it>
Orsetti
Valeria
V
Buratti
Emanuele
E
Baralle
Francisco
F
Cima
Valentina
V
Volpe
Marco
M
D'ascenzo
Carla
C
Palmieri
Arianna
A
Koutsikos
Kostantinos
K
Pegoraro
Elena
E
Angelini
Corrado
C
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*
D004268:DNA-Binding Proteins