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

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...

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Detalles Bibliográficos
Autores principales: Osmand, Alexander P., Bichell, Terry Jo., Bowman, Aaron B., Bates, Gillian P.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: IOS Press 2016
Materias:
Short Communication
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5181660/
https://www.ncbi.nlm.nih.gov/pubmed/27886014
http://dx.doi.org/10.3233/JHD-160217
Descripción
Sumario: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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