Calpain-mediated ataxin-3 cleavage in the molecular pathogenesis of spinocerebellar ataxia type 3 (SCA3)

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

(2012) A. T. Simoes et al.   BRAIN

N-terminal ataxin-3 causes neurological symptoms with inclusions, endoplasmic reticulum stress and ribosomal dislocation

(2011) Jeannette Hübener et al.   BRAIN

Calpain inhibitors: a survey of compounds reported in the patent and scientific literature

(2011) Isaac O Donkor   EXPERT OPINION ON THERAPEUTIC PATENTS

Photoreceptor rescue and toxicity induced by different calpain inhibitors

(2010) François Paquet-Durand et al.   JOURNAL OF NEUROCHEMISTRY

Preventing Ataxin-3 protein cleavage mitigates degeneration in a Drosophila model of SCA3

(2009) Joonil Jung et al.   HUMAN MOLECULAR GENETICS

Identification and functional dissection of localization signals within ataxin-3

(2009) Paul Michel Aloyse Antony et al.   NEUROBIOLOGY OF DISEASE

A transgenic mouse model of spinocerebellar ataxia type 3 resembling late disease onset and gender-specific instability of CAG repeats

(2009) Jana Boy et al.   NEUROBIOLOGY OF DISEASE

Calcium-bound structure of calpain and its mechanism of inhibition by calpastatin

(2008) Rachel A. Hanna et al.   NATURE

Study of subcellular localization and proteolysis of ataxin-3

(2008) Chiara Pozzi et al.   NEUROBIOLOGY OF DISEASE

Paradoxical delay in the onset of disease caused by super-long CAG repeat expansions in R6/2 mice

(2008) A Jennifer Morton et al.   NEUROBIOLOGY OF DISEASE

Intergenerational and striatal CAG repeat instability in Huntington's disease knock-in mice involve different DNA repair genes

(2008) Ella Dragileva et al.   NEUROBIOLOGY OF DISEASE