Embryonic Mutant Huntingtin Aggregate Formation in Mouse Models of Huntington's Disease.
J Huntingtons Dis
; 5(4): 343-346, 2016 12 15.
Article
en En
| MEDLINE
| ID: mdl-27886014
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.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Encéfalo
/
Enfermedad de Huntington
/
Proteína Huntingtina
Límite:
Animals
Idioma:
En
Revista:
J Huntingtons Dis
Año:
2016
Tipo del documento:
Article
País de afiliación:
Estados Unidos