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...

Descripción completa

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
_version_ 1782485741770637312
author Osmand, Alexander P.
Bichell, Terry Jo.
Bowman, Aaron B.
Bates, Gillian P.
author_facet Osmand, Alexander P.
Bichell, Terry Jo.
Bowman, Aaron B.
Bates, Gillian P.
author_sort Osmand, Alexander P.
collection PubMed
description 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.
format Online
Article
Text
id pubmed-5181660
institution National Center for Biotechnology Information
language English
publishDate 2016
publisher IOS Press
record_format MEDLINE/PubMed
spelling pubmed-51816602016-12-27 Embryonic Mutant Huntingtin Aggregate Formation in Mouse Models of Huntington’s Disease Osmand, Alexander P. Bichell, Terry Jo. Bowman, Aaron B. Bates, Gillian P. J Huntingtons Dis Short Communication 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. IOS Press 2016-12-15 /pmc/articles/PMC5181660/ /pubmed/27886014 http://dx.doi.org/10.3233/JHD-160217 Text en IOS Press and the authors. All rights reserved https://creativecommons.org/licenses/by-nc/4.0/ This is an open access article distributed under the terms of the Creative Commons Attribution Non-Commercial (CC BY-NC 4.0) License (https://creativecommons.org/licenses/by-nc/4.0/) , which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Short Communication
Osmand, Alexander P.
Bichell, Terry Jo.
Bowman, Aaron B.
Bates, Gillian P.
Embryonic Mutant Huntingtin Aggregate Formation in Mouse Models of Huntington’s Disease
title Embryonic Mutant Huntingtin Aggregate Formation in Mouse Models of Huntington’s Disease
title_full Embryonic Mutant Huntingtin Aggregate Formation in Mouse Models of Huntington’s Disease
title_fullStr Embryonic Mutant Huntingtin Aggregate Formation in Mouse Models of Huntington’s Disease
title_full_unstemmed Embryonic Mutant Huntingtin Aggregate Formation in Mouse Models of Huntington’s Disease
title_short Embryonic Mutant Huntingtin Aggregate Formation in Mouse Models of Huntington’s Disease
title_sort embryonic mutant huntingtin aggregate formation in mouse models of huntington’s disease
topic Short Communication
url 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
work_keys_str_mv AT osmandalexanderp embryonicmutanthuntingtinaggregateformationinmousemodelsofhuntingtonsdisease
AT bichellterryjo embryonicmutanthuntingtinaggregateformationinmousemodelsofhuntingtonsdisease
AT bowmanaaronb embryonicmutanthuntingtinaggregateformationinmousemodelsofhuntingtonsdisease
AT batesgillianp embryonicmutanthuntingtinaggregateformationinmousemodelsofhuntingtonsdisease

Ejemplares similares