Neural stem cells derived from the developing forebrain of YAC128 mice exhibit pathological features of Huntington's disease.

ABSTRACT:

Objectives

Huntington's disease (HD) is a devastating neurodegenerative disease caused by polyglutamine (polyQ) expansion in the huntingtin (HTT) gene. Mutant huntingtin (mHTT) is the main cause of HD and is associated with impaired mitochondrial dynamics, ubiquitin-proteasome system and autophagy, as well as tauopathy. In this study, we aimed to establish a new neural stem cell line for HD studies.

Materials and methods

YAC128 mice are a yeast artificial chromosome (YAC)-based transgenic mouse model of HD. These mice express a full-length human mutant HTT gene with 128 CAG repeats and exhibit various pathophysiological features of HD. In this study, we isolated a new neural stem cell line from the forebrains of YAC128 mouse embryos (E12.5) and analysed its characteristics u

SUBMITTER: Li E 

PROVIDER: S-EPMC7574873 | biostudies-literature | 2020 Oct

REPOSITORIES: biostudies-literature