Results 141 to 150 of about 13,262,735 (197)

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

, 2009
Protein cleavage is a common feature in human neurodegenerative disease. Ataxin-3 protein with an expanded polyglutamine (polyQ) repeat causes spinocerebellar ataxia type-3 (SCA3), also called Machado–Joseph disease, and is cleaved in mammalian cells ...
Derek Lessing, Joonil Jung, Kexiang Xu, Nancy M. Bonini   +3 more
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Polyglutamine-Expanded Ataxin-3 Accelerates CFTR Degradation Through K63-Linked Ubiquitination to Exacerbate Microglial Inflammation. [PDF]

ASN Neuro
Wang Z, Fan X, Bai B, Song M, Shi Y, Zhao Y, Xie P, Shi T, He C, Hu Y, Wu Q, Hou X.   +11 more
europepmc   +1 more source

Degron capability of the hydrophobic C-terminus of the polyglutamine disease protein, ataxin-3. [PDF]

J Neurosci Res, 2020
Blount JR, Johnson SL, Libohova K, Todi SV, Tsou WL.   +4 more
europepmc   +1 more source

Retrotransposition Events Shape the Evolution of the Ataxin-3 Gene Family in Primates. [PDF]

Genome Biol Evol
Felício D, Martins IM, Pinto A, Sequeiros J, Amorim A, Lopes AM, Seixas S, Martins S.   +7 more
europepmc   +1 more source

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. [PDF]

Front Mol Neurosci, 2021
Wiatr K, Marczak Ł, Pérot JB, Brouillet E, Flament J, Figiel M.   +5 more
europepmc   +1 more source

Ataxin-3 Overexpression via Adeno-associated Viral Vector Injection in the Primate Cerebellum: A Novel Model of Spinocerebellar Ataxia Type 3. [PDF]

Exp Neurobiol
Kim K, Kim A, Won J, Park J, Lim KS, Jeon CY, Min J, Cho JH, Song Y, Koo BS, Bae GS, Jeon E, Jeong KJ, Park SH, Lee HY, Choi WS, Lee DS, Lee Y.   +17 more
europepmc   +1 more source

Roles of orexinergic and noradrenergic neuronal activity in ketamine-induced sedation: a study using an orexin-ataxin-3 transgenic rat model. [PDF]

J Anesth
Tonosaki M, Kushikata T, Nikaido Y, Takekawa D, Kinoshita H, Saito J, Hirota K.   +6 more
europepmc   +1 more source

Evolutionary model of repeat insertions in Ataxin-3 traces the origin of the polyglutamine stretch to an ancestral ubiquitin binding module. [PDF]

Protein Sci
Felício D, Martins S, Alves GP, Amorim A, Macedo-Ribeiro S, Merski M.   +5 more
europepmc   +1 more source

The Machado-Joseph disease deubiquitylase ataxin-3 interacts with LC3C/GABARAP and promotes autophagy. [PDF]

Aging Cell, 2020
Herzog LK, Kevei É, Marchante R, Böttcher C, Bindesbøll C, Lystad AH, Pfeiffer A, Gierisch ME, Salomons FA, Simonsen A, Hoppe T, Dantuma NP.   +11 more
europepmc   +1 more source

Effects of the polyglutamine neurodegenerative protein ataxin-3 on transcriptional regulation and the ubiquitin -proteasome pathway

, 2003
Spinocerebellar ataxia type 3 (SCA3) results from expansion of a glutamine stretch in the disease protein, ataxin-3. SCA3 belongs to the polyglutamine disease family, for which the pathogenic mechanism remains elusive.
Li, Fusheng
core