Results 21 to 30 of about 14,352 (184)

Coaggregation of polyglutamine (polyQ) proteins is mediated by polyQ-tract interactions and impairs cellular proteostasis

Acta Biochimica et Biophysica Sinica, 2023
Nine polyglutamine (polyQ) proteins have already been identified that are considered to be associated with the pathologies of neurodegenerative disorders called polyQ diseases, but whether these polyQ proteins mutually interact and synergize in ...
Hong Jun-Ye, Wang Jian-Yang, Yue Hong-Wei, Zhang Xiang-Le, Zhang Shu-Xian, Jiang Lei-Lei, Hu Hong-Yu   +6 more
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Are Polyglutamine Diseases Expanding? [PDF]

Neuron, 2011
It remains a matter of speculation as to whether the sense CUG-containing RNA and/or the antisense CAG-encoding polyglutamine peptide serves as the pathogenic moiety in Huntington's disease like-2 (HDL2). In this issue of Neuron, Wilburn et al. show that in a HDL2 mouse model, the polyglutamine peptide drives disease progression.
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Activation of p38MAPK contributes to expanded polyglutamine-induced cytotoxicity.

PLoS ONE, 2008
BackgroundThe signaling pathways that may modulate the pathogenesis of diseases induced by expanded polyglutamine proteins are not well understood.Methodologies/principal findingsHerein we demonstrate that expanded polyglutamine protein cytotoxicity is ...
Maria Tsirigotis, R Mitchell Baldwin, Matthew Y Tang, Ian A J Lorimer, Douglas A Gray   +4 more
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Pathologic polyglutamine aggregation begins with a self-poisoning polymer crystal

eLife, 2023
A long-standing goal of amyloid research has been to characterize the structural basis of the rate-determining nucleating event. However, the ephemeral nature of nucleation has made this goal unachievable with existing biochemistry, structural biology ...
Tej Kandola, Shriram Venkatesan, Jiahui Zhang, Brooklyn T Lerbakken, Alex Von Schulze, Jillian F Blanck, Jianzheng Wu, Jay R Unruh, Paula Berry, Jeffrey J Lange, Andrew C Box, Malcolm Cook, Celeste Sagui, Randal Halfmann   +13 more
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Selective Destabilization of Transcripts by mRNA Decapping Regulates Oocyte Maturation and Innate Immunity Gene Expression during Ageing in C. elegans

Biology, 2023
Removal of the 5′ cap structure of RNAs (termed decapping) is a pivotal event in the life of cytoplasmic mRNAs mainly catalyzed by a conserved holoenzyme, composed of the catalytic subunit DCP2 and its essential cofactor DCP1.
Fivos Borbolis, Dimitra Ranti, Maria-Despina Papadopoulou, Sofia Dimopoulou, Apostolos Malatras, Ioannis Michalopoulos, Popi Syntichaki   +6 more
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The 11S Proteasomal Activator REGγ Impacts Polyglutamine-Expanded Androgen Receptor Aggregation and Motor Neuron Viability through Distinct Mechanisms

Frontiers in Molecular Neuroscience, 2017
Spinal and bulbar muscular atrophy (SBMA) is caused by expression of a polyglutamine (polyQ)-expanded androgen receptor (AR). The inefficient nuclear proteasomal degradation of the mutant AR results in the formation of nuclear inclusions containing amino-
Jill M. Yersak, Heather L. Montie, Erica S. Chevalier-Larsen, Yuhong Liu, Lan Huang, Martin Rechsteiner, Diane E. Merry   +6 more
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The role of ubiquitination in spinal and bulbar muscular atrophy

Frontiers in Molecular Neuroscience, 2022
Spinal and bulbar muscular atrophy (SBMA) is a neurodegenerative and neuromuscular genetic disease caused by the expansion of a polyglutamine-encoding CAG tract in the androgen receptor (AR) gene.
Medha Sengupta, Anna Pluciennik, Diane E. Merry   +2 more
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Autophagy and polyglutamine diseases

Progress in Neurobiology, 2012
In polyglutamine diseases, an abnormally elongated polyglutamine tract results in protein misfolding and accumulation of intracellular aggregates. The length of the polyglutamine expansion correlates with the tendency of the mutant protein to aggregate, as well as with neuronal toxicity and earlier disease onset.
Jimenez-Sanchez, Maria, Thomson, Frances, Zavodszky, Eszter, Rubinsztein, David C.   +3 more
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SWI/SNF senses carbon starvation with a pH-sensitive low-complexity sequence

eLife, 2022
It is increasingly appreciated that intracellular pH changes are important biological signals. This motivates the elucidation of molecular mechanisms of pH sensing.
J Ignacio Gutierrez, Gregory P Brittingham, Yonca Karadeniz, Kathleen D Tran, Arnob Dutta, Alex S Holehouse, Craig L Peterson, Liam J Holt   +7 more
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d-Polyglutamine Amyloid Recruits l-Polyglutamine Monomers and Kills Cells [PDF]

Journal of Molecular Biology, 2014
Polyglutamine (polyQ) amyloid fibrils are observed in disease tissue and have been implicated as toxic agents responsible for neurodegeneration in expanded CAG repeat diseases such as Huntington's disease. Despite intensive efforts, the mechanism of amyloid toxicity remains unknown. As a novel approach to probing polyQ toxicity, we investigate here how
Karunakar, Kar, Irene, Arduini, Kenneth W, Drombosky, Patrick C A, van der Wel, Ronald, Wetzel   +4 more
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