The Machado–Joseph disease‐associated form of ataxin‐3 impacts dynamics of clathrin‐coated pits
Sign Up to like & getrecommendations! Published in 2020 at "Cell Biology International"
DOI: 10.1002/cbin.11312
Abstract: Expansion above a certain threshold in the polyglutamine (polyQ) tract of ataxin‐3 is the main cause of neurodegeneration in Machado–Joseph disease. Ataxin‐3 contains an N‐terminal catalytic domain, called Josephin domain, and a highly aggregation‐prone C‐terminal… read more here.
Keywords: impacts dynamics; ataxin; machado joseph; dynamics clathrin ... See more keywords
LARGE‐PD: Examining the genetics of Parkinson's disease in Latin America
Sign Up to like & getrecommendations! Published in 2017 at "Movement Disorders"
DOI: 10.1002/mds.27081
Abstract: Expanded alleles accounted for 74.3% of the observed variability in the age at onset, whereas normal alleles explained a discrete additional 0.5% of the total age at onset variance, and 1.95% of the variance of… read more here.
Keywords: expanded alleles; wildtype; ataxin; genetics ... See more keywords
Mutant Ataxin-3-Containing Aggregates (MATAGGs) in Spinocerebellar Ataxia Type 3: Dynamics of the Disorder.
Sign Up to like & getrecommendations! Published in 2021 at "Molecular neurobiology"
DOI: 10.1007/s12035-021-02314-z
Abstract: Spinocerebellar ataxia type 3 (SCA3) is the most common type of SCA worldwide caused by abnormal polyglutamine expansion in the coding region of the ataxin-3 gene. Ataxin-3 is a multi-faceted protein involved in various cellular… read more here.
Keywords: ataxia type; spinocerebellar ataxia; ataxin containing; ataxin ... See more keywords
Dual-specificity phosphatase 18 modulates the SUMOylation and aggregation of Ataxin-1.
Sign Up to like & getrecommendations! Published in 2018 at "Biochemical and biophysical research communications"
DOI: 10.1016/j.bbrc.2018.05.178
Abstract: We previously reported that SUMOylation promotes the aggregation of ataxin-1 and JNK is involved in the process. Here we show that dual-specificity phosphatase 18 (DUSP18), a member of protein tyrosine phosphatases, exerts the opposite effects… read more here.
Keywords: aggregation; dual specificity; ataxin; aggregation ataxin ... See more keywords
Nuclear inclusions of pathogenic ataxin-1 induce oxidative stress and perturb the protein synthesis machinery
Sign Up to like & getrecommendations! Published in 2020 at "Redox Biology"
DOI: 10.1016/j.redox.2020.101458
Abstract: Spinocerebellar ataxia type-1 (SCA1) is caused by an abnormally expanded polyglutamine (polyQ) tract in ataxin-1. These expansions are responsible for protein misfolding and self-assembly into intranuclear inclusion bodies (IIBs) that are somehow linked to neuronal… read more here.
Keywords: protein; ataxin; stress; protein synthesis ... See more keywords
Capturing the Conformational Ensemble of the Mixed Folded Polyglutamine Protein Ataxin-3.
Sign Up to like & getrecommendations! Published in 2020 at "Structure"
DOI: 10.1016/j.str.2020.09.010
Abstract: Ataxin-3 is a deubiquitinase involved in protein quality control and other essential cellular functions. It preferentially interacts with polyubiquitin chains of four or more units attached to proteins delivered to the ubiquitin-proteasome system. Ataxin-3 is… read more here.
Keywords: protein; conformational ensemble; capturing conformational; ataxin ... See more keywords
Efficient Prevention of Neurodegenerative Diseases by Depletion of Starvation Response Factor Ataxin-2
Sign Up to like & getrecommendations! Published in 2017 at "Trends in Neurosciences"
DOI: 10.1016/j.tins.2017.06.004
Abstract: Ataxin-2 (ATXN2) homologs exist in all eukaryotic organisms and may have contributed to their origin. Apart from a role in endocytosis, they are known for global effects on mRNA repair and ribosomal translation. Cell size,… read more here.
Keywords: starvation; ataxin; efficient prevention; neurodegenerative diseases ... See more keywords
Therapeutic reduction of ataxin 2 extends lifespan and reduces pathology in TDP-43 mice
Sign Up to like & getrecommendations! Published in 2017 at "Nature"
DOI: 10.1038/nature22038
Abstract: Amyotrophic lateral sclerosis (ALS) is a rapidly progressing neurodegenerative disease that is characterized by motor neuron loss and that leads to paralysis and death 2–5 years after disease onset. Nearly all patients with ALS have… read more here.
Keywords: ataxin; tdp; pathology; therapeutic reduction ... See more keywords
Nuclear bodies formed by polyQ-ataxin-1 protein are liquid RNA/protein droplets with tunable dynamics
Sign Up to like & getrecommendations! Published in 2020 at "Scientific Reports"
DOI: 10.1038/s41598-020-57994-9
Abstract: A mutant form of the ataxin-1 protein with an expanded polyglutamine (polyQ) tract is the underlying cause of the inherited neurodegenerative disease spinocerebellar ataxia 1 (SCA1). In probing the biophysical features of the nuclear bodies… read more here.
Keywords: protein; ataxin protein; ataxin; polyq ataxin ... See more keywords
Autophagy mediates SUMO-induced degradation of a polyglutamine protein ataxin-3
Sign Up to like & getrecommendations! Published in 2017 at "Animal Cells and Systems"
DOI: 10.1080/19768354.2017.1330765
Abstract: ABSTRACT Previously, we reported that small ubiquitin-like modifier-1 (SUMO-1) promotes the degradation of a polyglutamine (polyQ) protein ataxin-3 and proposed that proteasomes mediate the proteolysis. Here, we present evidence that autophagy is also responsible for… read more here.
Keywords: protein; degradation; ataxin; sumo induced ... See more keywords
Ataxin‐1 controls the expression of specific noncoding RNAs in B cells upon autoimmune demyelination
Sign Up to like & getrecommendations! Published in 2023 at "Immunology and Cell Biology"
DOI: 10.1111/imcb.12622
Abstract: B cells play a key mechanistic role in the pathogenesis of multiple sclerosis (MS), a chronic neurological disease of the central nervous system with an autoimmune etiology. B cells contribute to disease initiation and progression… read more here.
Keywords: cell; disease; noncoding rnas; autoimmune demyelination ... See more keywords