Fragments of HdhQ150 mutant huntingtin form a soluble oligomer pool that declines with aggregate deposition upon aging.

doi:10.1371/journal.pone.0044457

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Article

Fragments of HdhQ150 mutant huntingtin form a soluble oligomer pool that declines with aggregate deposition upon aging.

PloS one - 1 Jan 2012

Marcellin David, Abramowski Dorothee, Young Douglas, Richter Jens, Weiss Andreas, Marcel Audrey, Maassen Julia, Kauffmann Muriel, Bibel Miriam, Shimshek Derya R, Faull Richard L M, Bates Gillian P, Kuhn Rainer R, Van der Putten P Herman, Schmid Peter, Lotz Gregor P

Abstract excerpt

Cleavage of the full-length mutant huntingtin (mhtt) protein into smaller, soluble aggregation-prone mhtt fragments appears to be a key process in the neuropathophysiology of Huntington's Disease (HD). Recent quantification studies using TR-FRET-based immunoassays showed decreasing levels of soluble mhtt correlating with an increased load of aggregated mhtt in the aging HdhQ150 mouse brain. To better characterize...

Topics

Aging
Animals
Brain
Chromatography
Disease Models, Animal
Embryonic Stem Cells
Fibroblasts
Fluorescence Resonance Energy Transfer
Humans
Huntingtin Protein

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Huntington Disease

Mice

Models, Biological

Mutation

Nerve Tissue Proteins

Protein Binding

Serotonin Plasma Membrane Transport Proteins

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Identifiers

PMID: 22984513
DOI: 10.1371/journal.pone.0044457
PMCID: PMC3440421

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