Calpastatin-mediated inhibition of calpains in the mouse brain prevents mutant ataxin 3 proteolysis, nuclear localization and aggregation, relieving Machado-Joseph disease

Brain

Volumn 135, Issue 8, 2012, Pages 2428-2439

  Simões, Ana T ^a,b   Gonçalves, Nélio ^a,b   Koeppen, Arnulf ^c   Déglon, Nicole ^d   Kügler, Sebastian ^e   Duarte, Carlos Bandeira ^a,f   Pereira De Almeida, Luís ^a,b  

^a UNIVERSITY OF COIMBRA   (Portugal)

^b UNIVERSITY OF COIMBRA   (Portugal)

^c STRATTON VA MEDICAL CENTER   (United States)

^d LAUSANNE UNIVERSITY HOSPITAL   (Switzerland)

^e UNIVERSITY OF GÖTTINGEN   (Germany)

^f UNIVERSITY OF COIMBRA   (Portugal)

Author keywords

calpastatin; cleavage fragment; Machado Joseph disease; proteolysis; spinocerebellar ataxia type 3

Indexed keywords

ATAXIN 3; CALPAIN; CALPASTATIN;

ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; COMPETITIVE INHIBITION; CONTROLLED STUDY; CORPUS STRIATUM; CYTOPLASM; DENTATE NUCLEUS; HUMAN; HUMAN CELL; HUMAN TISSUE; IMMUNOHISTOCHEMISTRY; LEFT HEMISPHERE; MACHADO JOSEPH DISEASE; MOUSE; NERVE DEGENERATION; NONHUMAN; PATHOGENESIS; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN DEGRADATION; PROTEIN DEPLETION; PROTEIN EXPRESSION; PROTEIN FUNCTION; PROTEIN LOCALIZATION; RIGHT HEMISPHERE; SPINOCEREBELLAR TRACT; WESTERN BLOTTING; WILD TYPE;

EID: 84864658791     PISSN: 00068950     EISSN: 14602156     Source Type: Journal    
DOI: 10.1093/brain/aws177     Document Type: Article

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