Deterioration of mitochondrial bioenergetics and ultrastructure impairment in skeletal muscle of a transgenic minipig model in the early stages of Huntington's disease

Deterioration of mitochondrial bioenergetics and ultrastructure impairment in skeletal muscle of a transgenic minipig model in the early stages of Huntington's disease

Skeletal muscle wasting and atrophy is one of the more severe clinical impairments resulting from the progression of Huntington's disease (HD). Mitochondrial dysfunction may play a significant role in the etiology of HD, but the specific condition of mitochondria in muscle has not been widely s...

Full description

Bibliographic Details
Main Authors: Marie Rodinova, Jana Krizova, Hana Stufkova, Bozena Bohuslavova, Georgina Askeland, Zaneta Dosoudilova, Stefan Juhas, Jana Juhasova, Zdenka Ellederova, Jiri Zeman, Lars Eide, Jan Motlik, Hana Hansikova
Format: Article
Language:English
Published: The Company of Biologists 2019-07-01
Series:Disease Models & Mechanisms
Subjects:
Huntington's disease
Mitochondrial function
Ultrastructure
HD large animal model
Disease development
Skeletal muscle
Biomarkers
Online Access:http://dmm.biologists.org/content/12/7/dmm038737

Internet

http://dmm.biologists.org/content/12/7/dmm038737

Similar Items