Results 51 to 60 of about 22,723 (239)
Polyglutamine Disease: Acetyltransferases Awry [PDF]
Current Biology, 2002 Recent evidence indicates that inhibition of histone acetyltransferases may be a primary cause of cellular pathogenesis in polyglutamine diseases such as Huntington disease; the results raise the possibility that pharmacologic manipulation of protein acetylation levels could be of therapeutic benefit.
openaire +2 more sources
Splice isoforms of the polyglutamine disease protein ataxin-3 exhibit similar enzymatic yet different aggregation properties. [PDF]
PLoS ONE, 2010 Protein context clearly influences neurotoxicity in polyglutamine diseases, but the contribution of alternative splicing to this phenomenon has rarely been investigated.
Ginny Marie Harris +4 more
doaj +1 more source
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
doaj +1 more source
PLoS ONE, 2009 BackgroundMisfolding- and aggregation-prone proteins underlying Parkinson's, Huntington's and Machado-Joseph diseases, namely alpha-synuclein, huntingtin, and ataxin-3 respectively, adopt numerous intracellular conformations during pathogenesis ...
Erik Kvam +5 more
doaj +1 more source
Ion channels and neuronal excitability in polyglutamine neurodegenerative diseases
Biomolecular Concepts, 2022 Polyglutamine (polyQ) diseases are a family composed of nine neurodegenerative inherited disorders (NDDs) caused by pathological expansions of cytosine-adenine-guanine (CAG) trinucleotide repeats which encode a polyQ tract in the corresponding proteins ...
Martinez-Rojas Vladimir A. +2 more
doaj +1 more source
Molecular Therapy: Nucleic Acids, 2021 Polyglutamine (polyQ) diseases are inherited neurodegenerative disorders caused by expansion of cytosine-adenine-guanine (CAG)-trinucleotide repeats in causative genes.
Tomoki Hirunagi +14 more
doaj +1 more source
The ubiquitin-proteasome pathway in Huntington's disease. [PDF]
, 2008 The accumulation of mutant protein is a common feature of neurodegenerative disease. In Huntington's disease, a polyglutamine expansion in the huntingtin protein triggers neuronal toxicity.
Finkbeiner, Steven, Mitra, Siddhartha
core +2 more sources
RACK1 modulates polyglutamine-induced neurodegeneration by promoting ERK degradation in Drosophila.
PLoS Genetics, 2021 Polyglutamine diseases are neurodegenerative diseases caused by the expansion of polyglutamine (polyQ) tracts within different proteins. Although multiple pathways have been found to modulate aggregation of the expanded polyQ proteins, the mechanisms by ...
Jun Xie, Yongchao Han, Tao Wang
doaj +1 more source
Changes in Purkinje cell firing and gene expression precede behavioral pathology in a mouse model of SCA2. [PDF]
, 2012 Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominantly inherited disorder, which is caused by a pathological expansion of a polyglutamine (polyQ) tract in the coding region of the ATXN2 gene.
Hansen, Stephen T +3 more
core +1 more source
Fighting polyglutamine disease by wrestling with SUMO [PDF]
Journal of Clinical Investigation, 2015 Spinobulbar muscular atrophy (SBMA) is an X-linked disease characterized by degeneration of motor neurons, muscle atrophy, and progressive weakness. It is caused by a polyglutamine (polyQ) expansion in the androgen receptor (AR), a transcription factor that is activated upon hormone binding.
Craig, Tim J, Henley, Jeremy M
openaire +3 more sources