Results 161 to 170 of about 13,262,735 (197)

Autophagy mediates SUMO-induced degradation of a polyglutamine protein ataxin-3. [PDF]

Anim Cells Syst (Seoul), 2017
Hwang SP, Lee DH.
europepmc   +1 more source

The Machado-Joseph Disease Deubiquitinase Ataxin-3 Regulates the Stability and Apoptotic Function of p53. [PDF]

PLoS Biol, 2016
Liu H, Li X, Ning G, Zhu S, Ma X, Liu X, Liu C, Huang M, Schmitt I, Wüllner U, Niu Y, Guo C, Wang Q, Tang TS.   +13 more
europepmc   +1 more source

Starvation-induced HSC70 O-GlcNAcylation activates chaperone-mediated autophagy. [PDF]

J Biol Chem
Xu N, Wang X, Zhang J, Zhao J, Yan S, Zhou W, Chi W, Li J.   +7 more
europepmc   +1 more source

Oligodendrocyte dysfunction contributes to motor deficits and Purkinje cell axonopathy in spinocerebellar ataxia type 1. [PDF]

J Clin Invest
Lee C, Grijalva RM, Tejwani L, Bae E, Chase A, Ro H, Kim H, Olmos V, Orengo JP, Lim J.   +9 more
europepmc   +1 more source

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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, Xiaochuan Ma, Hongfeng Wang   +2 more
exaly   +3 more sources

Structural modeling of ataxin‐3 reveals distant homology to adaptins

Proteins: Structure, Function, and Bioinformatics, 2002
AbstractSpinocerebellar ataxia type 3 (SCA3) is a polyglutamine disorder caused by a CAG repeat expansion in the coding region of a gene encoding ataxin‐3, a protein of yet unknown function. Based on a comprehensive computational analysis, we propose a structural model and structure‐based functions for ataxin‐3.
Albrecht, Mario, Hoffmann, Daniel, Evert, Bernd O., Schmitt, Ina, Wüllner, Ullrich, Lengauer, Thomas   +5 more
openaire   +5 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, J Q, Trojanowski, D G, Leonard, K L, Chen, J L, Barnett, J B, Leverenz, T D, Bird, Y, Robitaille, A, Malandrini, K H, Fischbeck   +9 more
openaire   +4 more sources

Biophysical Characterisation of the Josephin Domain of Ataxin-3

, 2017
The misfolding and subsequent aggregation of proteins underpins a large number of conditions called conformational diseases. Many of these diseases are caused by genetic mutations in protein coding regions. One such family is the polyglutamine diseases, characterised by the expansion of a naturally occurring glutamine repeat beyond a specific threshold
Christopher Lupton (3728302)
openaire   +2 more sources

Unraveling the Role of Ataxin-2 in Metabolism

Trends in Endocrinology and Metabolism, 2017
Ataxin-2 is a polyglutamine protein implicated in several biological processes such as RNA metabolism and cytoskeleton reorganization. Ataxin-2 is highly expressed in various tissues including the hypothalamus, a brain region that controls food intake ...
Sara Carmo-Silva, Clévio Nóbrega, Luis Pereira De Almeida   +2 more
exaly   +2 more sources