Results 11 to 20 of about 30,328 (180)
Suppression of polyglutamine toxicity by a Drosophila homolog of myeloid leukemia factor 1 [PDF]
, 2002 The toxicity of an abnormally long polyglutamine [poly(Q)] tract within specific proteins is the molecular lesion shared by Huntington's disease (HD) and several other hereditary neurodegenerative disorders.
Benzer, Seymour, Kazemi-Esfarjani, Parsa
core +1 more source
Brain Sciences, 2014 Huntington disease and other diseases of polyglutamine expansion are each caused by a different protein bearing an excessively long polyglutamine sequence and are associated with neuronal death.
Guylaine Hoffner, Philippe Djian
doaj +1 more source
Oxidative Stress and Neurodegeneration: Interconnected Processes in PolyQ Diseases
Antioxidants, 2021 Neurodegenerative polyglutamine (polyQ) disorders are caused by trinucleotide repeat expansions within the coding region of disease-causing genes. PolyQ-expanded proteins undergo conformational changes leading to the formation of protein inclusions which
Ioannis Gkekas +5 more
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Na+/H+ exchangers induce autophagy in neurons and inhibit polyglutamine-induced aggregate formation.
PLoS ONE, 2013 In polyglutamine diseases, an abnormally elongated polyglutamine results in protein misfolding and accumulation of intracellular aggregates. Autophagy is a major cellular degradative pathway responsible for eliminating unnecessary proteins, including ...
Kazuya Togashi +5 more
doaj +1 more source
J Proteins Counteract Amyloid Propagation and Toxicity in Yeast
Biology, 2022 The accumulation of misfolded proteins as amyloids is associated with pathology in dozens of debilitating human disorders, including diabetes, Alzheimer’s, Parkinson’s, and Huntington’s diseases.
Daniel C. Masison +2 more
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Molecular Mechanisms in Pentanucleotide Repeat Diseases
Cells, 2022 The number of neurodegenerative diseases resulting from repeat expansion has increased extraordinarily in recent years. In several of these pathologies, the repeat can be transcribed in RNA from both DNA strands producing, at least, one toxic RNA repeat ...
Joana R. Loureiro +3 more
doaj +1 more source
A Variable Polyglutamine Repeat Affects Subcellular Localization and Regulatory Activity of a Populus ANGUSTIFOLIA Protein. [PDF]
, 2018 Polyglutamine (polyQ) stretches have been reported to occur in proteins across many organisms including animals, fungi and plants. Expansion of these repeats has attracted much attention due their associations with numerous human diseases including ...
Barry, Kerrie +12 more
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Frontiers in Cellular Neuroscience, 2022 Huntington disease (HD) is caused by the expansion of CAG triplet repeats in exon 1 of the huntingtin (HTT) gene, which also encodes the first 17 amino acids (N-17) that can modulate the toxicity of the expanded polyQ repeat. N-17 are conserved in a wide
Xianxian Zhao +5 more
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eLife, 2016 The polyglutamine expansion in huntingtin protein causes Huntington’s disease. Here, we investigated structural and biochemical properties of huntingtin and the effect of the polyglutamine expansion using various biophysical experiments including ...
Ravi Vijayvargia +13 more
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Pharmaceutics, 2021 Seven of the most frequent spinocerebellar ataxias (SCAs) are caused by a pathological expansion of a cytosine, adenine and guanine (CAG) trinucleotide repeat located in exonic regions of unrelated genes, which in turn leads to the synthesis of ...
Fabiola V. Borbolla-Jiménez +5 more
doaj +1 more source