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Phosphorylation of ataxin-3 by glycogen synthase kinase 3β at serine 256 regulates the aggregation of ataxin-3

Biochemical and Biophysical Research Communications, 2007
Machado-Joseph disease (MJD) is a dominant neurodegenerative disorder caused by an expansion of the polyglutamine tract in MJD-1 gene product, ataxin-3. Recently, studies show that phosphorylation of polyglutamine disease proteins, such as huntingtin, ataxin-1 and DRPLA, plays an important role in regulating pathogenesis.
Erkang Fei   +2 more
exaly   +3 more sources

Molecular clearance of ataxin-3 is regulated by a mammalian E4 [PDF]

open access: yesEMBO Journal, 2004
Insoluble aggregates of polyglutamine-containing proteins are usually conjugated with ubiquitin in neurons of individuals with polyglutamine diseases. We now show that ataxin-3, in which the abnormal expansion of a polyglutamine tract is responsible for spinocerebellar ataxia type 3 (SCA3), undergoes ubiquitylation and degradation by the proteasome ...
Masaki Matsumoto   +2 more
exaly   +3 more sources

Temperature profoundly affects ataxin-3 fibrillogenesis

Biochimie, 2012
Ataxin-3 (AT3) triggers spinocerebellar ataxia type 3 when it carries a polyglutamine stretch expanded beyond a critical threshold. By Fourier transform infrared spectroscopy and atomic force microscopy we previously showed that a normal (AT3Q24) and an expanded (AT3Q55) variant were capable of evolving into oligomers and protofibrils at 37 °C, whereas
A. Apicella   +8 more
openaire   +3 more sources

Structural and functional analysis of ataxin‐2 and ataxin‐3

European Journal of Biochemistry, 2004
Spinocerebellar ataxia types 2 (SCA2) and 3 (SCA3) are autosomal‐dominantly inherited, neurodegenerative diseases caused by CAG repeat expansions in the coding regions of the genes encoding ataxin‐2 and ataxin‐3, respectively. To provide a rationale for further functional experiments, we explored the protein architectures of ataxin‐2 and ataxin‐3 ...
Albrecht, M.   +3 more
openaire   +3 more sources

Aggregation of the polyglutamine protein ataxin-3

2021
This thesis was scanned from the print manuscript for digital preservation and is copyright the author. Researchers can access this thesis by asking their local university, institution or public library to make a request on their behalf. Monash staff and postgraduate students can use the link in the References field.
openaire   +1 more source

Dynamic expression of Hsp27 in the presence of mutant ataxin-3

Biochemical and Biophysical Research Communications, 2005
Machado-Joseph disease (MJD)/spinocerebellar ataxia type 3 (SCA3) is an autosomal dominant spinocerebellar degeneration characterized by a wide range of clinical manifestations. The molecular mechanisms underlying the selective neuronal death typical of MJD/SCA3 are unknown.
Wei-Hsiu, Chang   +8 more
openaire   +2 more sources

Ataxin 1 and ataxin 3 in neuronal intranuclear inclusion disease

Annals of Neurology, 1999
Neuronal intranuclear inclusion disease (NIID) is a multisystem neurodegenerative disorder characterized by large intranuclear aggregates in neurons of the central and peripheral nervous system. These ubiquitinated intranuclear inclusions are morphologically similar to the intraneuronal aggregates that have been identified in the CAG/polyglutamine ...
A P, Lieberman   +9 more
openaire   +3 more sources

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