Polyglutamine tract vs. protein context in SCA1 pathogenesis

2020 ◽  
pp. 105-117
Author(s):  
Harry T. Orr ◽  
Huda Y. Zoghbi
Keyword(s):  
Polyglutamine Tract

scholarly journals The folding equilibrium of huntingtin exon 1 monomer depends on its polyglutamine tract

2018 ◽  
Vol 293 (51) ◽  
pp. 19613-19623 ◽  
Author(s):  
Jose M. Bravo-Arredondo ◽  
Natalie C. Kegulian ◽  
Thomas Schmidt ◽  
Nitin K. Pandey ◽  
Alan J. Situ ◽  
...  
Keyword(s):  
Exon 1 ◽  
Polyglutamine Tract

Differences in activation of MAP kinases and variability in the polyglutamine tract of Slt2 in clinical and non-clinical isolates of Saccharomyces cerevisiae

Yeast ◽  
2010 ◽  
Vol 27 (8) ◽  
pp. 549-561 ◽  
Author(s):  
Rosa de Llanos ◽  
Carolina Hernández-Haro ◽  
Eladio Barrio ◽  
Amparo Querol ◽  
María Teresa Fernández-Espinar ◽  
...  
Keyword(s):  
Saccharomyces Cerevisiae ◽  
Map Kinases ◽  
Clinical Isolates ◽  
Polyglutamine Tract

scholarly journals Association of Moderate Polyglutamine Tract Expansions in the Slow Calcium-Activated Potassium Channel Type 3 With Ataxia

2001 ◽  
Vol 58 (10) ◽  
pp. 1649 ◽  
Author(s):  
Karla Patricia Figueroa ◽  
Piu Chan ◽  
Ludger Schöls ◽  
Carline Tanner ◽  
Olaff Riess ◽  
...  
Keyword(s):  
Potassium Channel ◽  
Polyglutamine Tract ◽  
Calcium Activated Potassium Channel ◽  
Type 3 ◽  
Channel Type

scholarly journals Bim contributes to the progression of Huntington’s disease-associated phenotypes

2019 ◽  
Vol 29 (2) ◽  
pp. 216-227
Author(s):  
Sheridan L Roberts ◽  
Tracey Evans ◽  
Yi Yang ◽  
Yuhua Fu ◽  
Robert W Button ◽  
...  
Keyword(s):  
Mouse Model ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Neuronal Death ◽  
Neurodegenerative Disorder ◽  
Neuronal Degeneration ◽  
Brain Regions ◽  
Post Mortem ◽  
Polyglutamine Tract

Abstract Huntington’s disease (HD) is a neurodegenerative disorder caused by an expanded polyglutamine tract in the huntingtin (HTT) protein. Mutant HTT (mHTT) toxicity is caused by its aggregation/oligomerization. The striatum is the most vulnerable region, although all brain regions undergo neuronal degeneration in the disease. Here we show that the levels of Bim, a BH3-only protein, are significantly increased in HD human post-mortem and HD mouse striata, correlating with neuronal death. Bim reduction ameliorates mHTT neurotoxicity in HD cells. In the HD mouse model, heterozygous Bim knockout significantly mitigates mHTT accumulation and neuronal death, ameliorating disease-associated phenotypes and lifespan. Therefore, Bim could contribute to the progression of HD.

scholarly journals Androgen receptor with elongated polyglutamine tract forms aggregates that alter axonal trafficking and mitochondrial distribution in motoneuronal processes

2002 ◽  
Vol 16 (11) ◽  
pp. 1418-1420 ◽  
Author(s):  
Federica Piccioni ◽  
Paolo Pinton ◽  
Silvia Simeoni ◽  
Paola Pozzi ◽  
Umberto Fascio ◽  
...  
Keyword(s):  
Androgen Receptor ◽  
Mitochondrial Distribution ◽  
Polyglutamine Tract ◽  
Axonal Trafficking

scholarly journals Caspase Cleavage of Gene Products Associated with Triplet Expansion Disorders Generates Truncated Fragments Containing the Polyglutamine Tract

1998 ◽  
Vol 273 (15) ◽  
pp. 9158-9167 ◽  
Author(s):  
Cheryl L. Wellington ◽  
Lisa M. Ellerby ◽  
Abigail S. Hackam ◽  
Russell L. Margolis ◽  
Mark A. Trifiro ◽  
...  
Keyword(s):  
Gene Products ◽  
Caspase Cleavage ◽  
Triplet Expansion ◽  
Polyglutamine Tract

Endoplasmic reticulum chaperone GRP78 suppresses the aggregation of proteins containing expanded polyglutamine tract

2012 ◽  
Vol 422 (3) ◽  
pp. 527-533 ◽  
Author(s):  
Nobuyuki Yamagishi ◽  
Shoichi Magara ◽  
Satoko Tamura ◽  
Youhei Saito ◽  
Takumi Hatayama
Keyword(s):  
Endoplasmic Reticulum ◽  
Endoplasmic Reticulum Chaperone ◽  
Polyglutamine Tract

PQBP-1 (Np/PQ): a polyglutamine tract-binding and nuclear inclusion-forming protein

2001 ◽  
Vol 56 (3-4) ◽  
pp. 273-280 ◽  
Author(s):  
Hitoshi Okazawa ◽  
Marius Sudol ◽  
Tina Rich
Keyword(s):  
Nuclear Inclusion ◽  
Polyglutamine Tract
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