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Updated: Mar 11, 2026
Fractionation for Resolution of Soluble and Insoluble Huntingtin Species
Published on: February 27, 2018
Embryonic Mutant Huntingtin Aggregate Formation in Mouse Models of Huntington's Disease
Alexander P Osmand1, Terry Jo Bichell2, Aaron B Bowman3
1Department of Biochemistry and Cellular and Molecular Biology, University of Tennessee, Knoxville, TN, USA.
Abstract:
The role of aggregate formation in the pathophysiology of Huntington's disease (HD) remains uncertain. However, the temporal appearance of aggregates tends to correlate with the onset of symptoms and the numbers of neuropil aggregates correlate with the progression of clinical disease. Using highly sensitive immunohistochemical methods we have detected the appearance of diffuse aggregates during embryonic development in the R6/2 and YAC128 mouse models of HD. These are initially seen in developing axonal tracts and appear to spread throughout the cerebrum in the early neonate.
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