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Hunting for huntingtin associated factors: Identification and characterization of huntingtin expanded polyglutamine aggregate associated factors and their impact on Huntington disease model cellular toxicity
Uniformed Services University of the Health Sciences Bethesda United States
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Gene mutations resulting in the formation of insoluble protein aggregates, or amyloid-like structures, have been correlated with a number of pathological conditions. Huntingtons disease HD is one of the most prevalent neurodegenerative diseases, characterized by movement, memory, behavioral, and cognitive difficulties, which become more severe as the disease progresses. Protein aggregation in HD is caused by expansion of the CAG repeat tract, also known as a polyglutamine polyQ expansion, in exon one of the Huntingtin protein Htt. Research has shown that Huntingtin polyQ HttpolyQ mutant expansions beyond 35 repeats result in protein aggregation. These HttpolyQ aggregates form amyloid-like inclusions characterized by cross-beta structure and insolubility and can result in loss of Huntingtin protein function or sequestration of essential cellular proteins that tightly interact with the aggregate.
APPROVED FOR PUBLIC RELEASE