Article
A cellular model that recapitulates major pathogenic steps of Huntington's disease.
Human molecular genetics - 1 Sept 1998
Lunkes A, Mandel J L
Abstract excerpt
To gain insight into the pathogenic mechanisms of Huntington's disease (HD), we have developed a stable cellular model, using a neuroblastoma cell line in which the expression of full-length or truncated forms of wild-type and mutant huntingtin can be induced. While the wild-type forms have the expected cytoplasmic localization, the expression of mutant proteins leads to the formation of cytoplasmic and nuclear...
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