[HTML][HTML] Chronic treatment with 17-DMAG improves balance and coordination in a new mouse model of Machado-Joseph disease
A Silva-Fernandes, S Duarte-Silva, A Neves-Carvalho… - …, 2014 - Elsevier
Machado-Joseph disease (MJD) or spinocerebellar ataxia type 3 (SCA3) is a
neurodegenerative disease currently with no treatment. We describe a novel mouse model
of MJD which expresses mutant human ataxin-3 at near endogenous levels and manifests
MJD-like motor symptoms that appear gradually and progress over time. CMVMJD135 mice
show ataxin-3 intranuclear inclusions in the CNS and neurodegenerative changes in key
disease regions, such as the pontine and dentate nuclei. Hsp90 inhibition has shown …
neurodegenerative disease currently with no treatment. We describe a novel mouse model
of MJD which expresses mutant human ataxin-3 at near endogenous levels and manifests
MJD-like motor symptoms that appear gradually and progress over time. CMVMJD135 mice
show ataxin-3 intranuclear inclusions in the CNS and neurodegenerative changes in key
disease regions, such as the pontine and dentate nuclei. Hsp90 inhibition has shown …