Enhanced Molecular Mobility of Ordinarily Structured Regions Drives Polyglutamine Disease*
Background: Polyglutamine tract expansion within disease-associated proteins leads to protein aggregation and disease. Results: Molecular mobility of a single α-helix within ataxin-3 positively scales with polyglutamine tract length and drives misfolding
C. Lupton+5 more
semanticscholar +1 more source
The Social Amoeba Dictyostelium discoideum Is Highly Resistant to Polyglutamine Aggregation*
Background: The Dictyostelium proteome is predicted to encode a vast amount of homopolymeric amino acid tracts, including long polyglutamine tracts. Results: Proteins with long polyglutamine tracts are soluble in Dictyostelium.
Stephanie Santarriaga+6 more
semanticscholar +1 more source
Adenovirus-mediated expression of mutant DRPLA proteins with expanded polyglutamine stretches in neuronally differentiated PC12 cells. Preferential intranuclear aggregate formation and apoptosis [PDF]
Toshiya Sato
openalex +1 more source
Background: Polyglutamine-expanded androgen receptor forms nuclear inclusions of cleaved AR. Results: Soluble aggregates and insoluble inclusions of polyglutamine-expanded AR contain full-length AR, which becomes proteolyzed by the proteasome within ...
E. Heine+5 more
semanticscholar +1 more source
Evidence for Proteasome Involvement in Polyglutamine Disease: Localization to Nuclear Inclusions in SCA3/MJD and Suppression of Polyglutamine Aggregation in vitro [PDF]
Y. Chai+4 more
openalex +1 more source
Pathogenesis of SCA3 and implications for other polyglutamine diseases
Hayley S. McLoughlin+2 more
semanticscholar +1 more source
A genetic model for human polyglutamine-repeat disease in Drosophila melanogaster [PDF]
Nancy M. Bonini
openalex +1 more source
Neurodegeration based on polyglutamine aggregation
One of the major hypotheses about polyQ toxicity is the sequestration of functionally important proteins into the aggregates. We established and carried out a direct, systematic proteomic analysis of aggregate-interacting proteins (AIPs). This analysis, as well as other studies in our lab, has revealed the following AIPs in addition to our previously ...
openaire +3 more sources
Cytoplasmic Localization and the Choice of Ligand Determine Aggregate Formation by Androgen Receptor with Amplified Polyglutamine Stretch [PDF]
Matthias Becker+4 more
openalex +1 more source
Mechanisms of chaperone suppression of polyglutamine disease: selectivity, synergy and modulation of protein solubility in Drosophila [PDF]
Ho Yin Edwin Chan+4 more
openalex +1 more source