Results 31 to 40 of about 14,329 (183)
Solution structure of polyglutamine tracts in GST‐polyglutamine fusion proteins
FEBS Letters, 2002 Aggregation of expanded polyglutamine (polyQ) seems to be the cause of various genetic neurodegenerative diseases. Relatively little is known as yet about the polyQ structure and the mechanism that induces aggregation. We have characterised the solution structure of polyQ in a proteic context using a model system based on glutathione S‐transferase ...
Masino L +5 more
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Cells, 2022 Emerging evidence has implicated non-neuronal cells, particularly oligodendrocytes, in the pathophysiology of many neurodegenerative diseases, including Alzheimer’s disease, Parkinson’s disease, amyotrophic lateral sclerosis, Huntington’s disease and ...
Kristen H. Schuster +2 more
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Frontiers in Neuroscience, 2022 Microsatellites are repetitive sequences commonly found in the genomes of higher organisms. These repetitive sequences are prone to expansion or contraction, and when microsatellite expansion occurs in the regulatory or coding regions of genes this can ...
Felicia N. Williams +3 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 +4 more
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Biomedicines, 2023 Brain-derived neurotrophic factor (BDNF) is a major neurotrophin whose loss or interruption is well established to have numerous intersections with the pathogenesis of progressive neurological disorders.
Andrew Speidell +2 more
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Biomedicines, 2021 Polyglutamine (polyQ) ataxias are a heterogenous group of neurological disorders all caused by an expanded CAG trinucleotide repeat located in the coding region of each unique causative gene.
Craig S. McIntosh +3 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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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 +4 more
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Biochemistry Research International, 2012 Huntington’s disease is a progressive neurodegenerative disease, caused by a polyglutamine expansion in the huntingtin protein. A prominent hallmark of the disease is the presence of intracellular aggregates initiated by N-terminal huntingtin fragments ...
Sabine Schipper-Krom +2 more
doaj +1 more source