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

Osmand AP, Bichell TJ, Bowman AB, Bates GP
J Huntingtons Dis. 2016 5 (4): 343-346

PMID: 27886014 · PMCID: PMC5181660 · DOI:10.3233/JHD-160217

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.

MeSH Terms (9)

Animals Axons Brain Disease Models, Animal Huntingtin Protein Huntington Disease Immunohistochemistry Mice, Transgenic Protein Aggregation, Pathological

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