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Publication Detail

Title: Embryonic Mutant Huntingtin Aggregate Formation in Mouse Models of Huntington's Disease.

Authors: Osmand, Alexander P; Bichell, Terry Jo; Bowman, Aaron B; Bates, Gillian P

Published In J Huntingtons Dis, (2016 Dec 15)

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.

PubMed ID: 27886014 Exiting the NIEHS site

MeSH Terms: Animals; Axons/metabolism; Axons/pathology; Brain/embryology*; Brain/metabolism*; Brain/pathology; Disease Models, Animal; Huntingtin Protein/metabolism*; Huntington Disease/embryology*; Huntington Disease/metabolism*; Huntington Disease/pathology; Immunohistochemistry; Mice, Transgenic; Protein Aggregation, Pathological/embryology; Protein Aggregation, Pathological/metabolism; Protein Aggregation, Pathological/pathology

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