Astrocytes generated from patient induced pluripotent stem cells recapitulate features of Huntington's disease patient cells.

ABSTRACT:

Background

Huntington's Disease (HD) is a devastating neurodegenerative disorder that clinically manifests as motor dysfunction, cognitive impairment and psychiatric symptoms. There is currently no cure for this progressive and fatal disorder. The causative mutation of this hereditary disease is a trinucleotide repeat expansion (CAG) in the Huntingtin gene that results in an expanded polyglutamine tract. Multiple mechanisms have been proposed to explain the preferential striatal and cortical degeneration that occurs with HD, including non-cell-autonomous contribution from astrocytes. Although numerous cell culture and animal models exist, there is a great need for experimental systems that can more accurately replicate the human disease. Human induced pluripotent stem cells (iPSCs)

SUBMITTER: Juopperi TA 

PROVIDER: S-EPMC3506453 | biostudies-literature | 2012 May

REPOSITORIES: biostudies-literature