Results 11 to 20 of about 14,329 (183)
Proteasome degrades soluble expanded polyglutamine completely and efficiently
Neurobiology of Disease, 2004 To date, nine progressive neurodegenerative diseases are caused by expansion of the CAG repeat coding for polyglutamine, including Huntington's disease and several forms of spinocerebellar ataxia.
Christine Van Broeckhoven
exaly +3 more sources
Biomolecules, 2022 Nine heritable diseases are known that are caused by unphysiologically elongated polyglutamine tracts in human proteins leading to misfolding, aggregation and neurodegeneration.
Pauline Elisabeth Kolkwitz +2 more
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
doaj +1 more source
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
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
doaj +1 more source
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
Novel polyglutamine model uncouples proteotoxicity from aging. [PDF]
PLoS ONE, 2014 Polyglutamine expansions in certain proteins are the genetic determinants for nine distinct progressive neurodegenerative disorders and resultant age-related dementia.
Nakeirah T M Christie +4 more
doaj +1 more source
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
doaj +1 more source
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
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 +6 more
doaj +1 more source