Mutant Ataxin-3-Containing Aggregates (MATAGGs) in Spinocerebellar Ataxia Type 3: Dynamics of the Disorder.
Sign Up to like & getrecommendations! Published in 2021 at "Molecular neurobiology"
DOI: 10.1007/s12035-021-02314-z
Abstract: Spinocerebellar ataxia type 3 (SCA3) is the most common type of SCA worldwide caused by abnormal polyglutamine expansion in the coding region of the ataxin-3 gene. Ataxin-3 is a multi-faceted protein involved in various cellular… read more here.
Keywords: ataxia type; spinocerebellar ataxia; ataxin containing; ataxin ... See more keywords
Caffeic acid and resveratrol ameliorate cellular damage in cell and Drosophila models of spinocerebellar ataxia type 3 through upregulation of Nrf2 pathway
Sign Up to like & getrecommendations! Published in 2018 at "Free Radical Biology and Medicine"
DOI: 10.1016/j.freeradbiomed.2017.12.011
Abstract: ABSTRACT Polyglutamine (polyQ)‐expanded mutant ataxin‐3 protein, which is prone to misfolding and aggregation, leads to cerebellar neurotoxicity in spinocerebellar ataxia type 3 (SCA3), an inherited PolyQ neurodegenerative disease. Although the exact mechanism is unknown, the… read more here.
Keywords: drosophila; ataxia type; spinocerebellar ataxia; sca3 ... See more keywords
Broad Influence of Mutant Ataxin-3 on the Proteome of the Adult Brain, Young Neurons, and Axons Reveals Central Molecular Processes and Biomarkers in SCA3/MJD Using Knock-In Mouse Model
Sign Up to like & getrecommendations! Published in 2021 at "Frontiers in Molecular Neuroscience"
DOI: 10.3389/fnmol.2021.658339
Abstract: Spinocerebellar ataxia type 3 (SCA3/MJD) is caused by CAG expansion mutation resulting in a long polyQ domain in mutant ataxin-3. The mutant protein is a special type of protease, deubiquitinase, which may indicate its prominent… read more here.
Keywords: sca3 mjd; sca3; model; mutant ataxin ... See more keywords
Mutant Ataxin-2 Expression in Aged Animals Aggravates Neuropathological Features Associated with Spinocerebellar Ataxia Type 2
Sign Up to like & getrecommendations! Published in 2022 at "International Journal of Molecular Sciences"
DOI: 10.3390/ijms231911896
Abstract: Spinocerebellar ataxia type 2 (SCA2) is a rare autosomal, dominantly inherited disease, in which the affected individuals have a disease onset around their third life decade. The molecular mechanisms underlying SCA2 are not yet completely… read more here.
Keywords: mutant ataxin; neuropathological features; ataxia type; spinocerebellar ataxia ... See more keywords