Results 1 to 10 of about 30,309 (161)
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
Filter Retardation Assay for Detecting and Quantifying Polyglutamine Aggregates Using Caenorhabditis elegans Lysates [PDF]
, 2018 Protein aggregation is a hallmark of several neurodegenerative diseases and is associated with impaired protein homeostasis. This imbalance is caused by the loss of the protein's native conformation, which ultimately results in its aggregation or ...
Mata Cabana, Alejandro +3 more
core +14 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
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
Frontiers in Molecular Biosciences, 2023 Polyglutamine expansion at the N-terminus of the huntingtin protein exon 1 (Htt-ex1) is closely associated with a number of neurodegenerative diseases, which result from the aggregation of the increased polyQ repeat.
Mohammed Khaled +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
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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
doaj +1 more source
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
doaj +1 more source
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
doaj +1 more source
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
doaj +1 more source