Results 21 to 30 of about 21,037 (206)
Polyglutamine Repeats in Viruses [PDF]
Molecular Neurobiology, 2018 This review explores the presence and functions of polyglutamine (polyQ) in viral proteins. In mammals, mutations in polyQ segments (and CAG repeats at the nucleotide level) have been linked to neural disorders and ataxias. PolyQ regions in normal human proteins have documented functional roles, in transcription factors and, more recently, in ...
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Solubilization and disaggregation of polyglutamine peptides [PDF]
Protein Science, 2001 AbstractA method is described for dissolving and disaggregating chemically synthesized polyglutamine peptides. Polyglutamine peptides longer than about Q20 have been reported to be insoluble in water, but dissolution in – and evaporation from ‐ a mixture of trifluoroacetic acid and hexafluoroisopropanol converts polyglutamine peptides up to at least ...
Songming Chen, Ronald Wetzel
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Hosting Neurotoxicity in Polyglutamine Disease [PDF]
Cell, 2006 Polyglutamine diseases are caused by an expanded glutamine domain thought to confer a toxic activity onto the respective disease proteins. In this issue, propose that toxicity of the polyglutamine protein Ataxin-1 may not be due to abberant protein interactions mediated by the polyglutamine expansion.
Nancy M. Bonini +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 +3 more
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The Missing Link in Polyglutamine Diseases [PDF]
Movement Disorders Clinical Practice, 2017 Polyglutamine diseases are a group of nine hereditary neurodegenerative disorders and include Huntington disease (HD), the most prevalent spinocerebellar ataxias (SCAs type 1, 2, 3, 6, 7 and 17), dentatorubral-pallidoluysian atrophy and spinal and bulbar muscular atrophy (also known as Kennedy disease).
Bettina Balint +3 more
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Polyglutamine tracts regulate beclin 1-dependent autophagy
Nature, 2017 Nine neurodegenerative diseases are caused by expanded polyglutamine (polyQ) tracts in different proteins, such as huntingtin in Huntington’s disease and ataxin 3 in spinocerebellar ataxia type 3 (SCA3).
A. Ashkenazi +10 more
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eLife, 2020 Spinocerebellar ataxia type 3 (SCA3) belongs to the family of polyglutamine neurodegenerations. Each disorder stems from the abnormal lengthening of a glutamine repeat in a different protein. Although caused by a similar mutation, polyglutamine disorders
Sean L. Johnson +4 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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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
Irene Arduini +4 more
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Genetics, Mechanisms, and Therapeutic Progress in Polyglutamine Spinocerebellar Ataxias
Neurotherapeutics, 2019 Autosomal dominant cerebellar ataxias (ADCAs) are a group of neurodegenerative disorders characterized by degeneration of the cerebellum and its connections.
R. Buijsen +3 more
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