Article
C-terminal deletion of the atrophin-1 protein results in growth retardation but not neurodegeneration in mice.
Developmental dynamics : an official publication of the American Association of Anatomists - 1 Oct 2009
Yu Juehua, Ying Mingyao, Zhuang Yuan, Xu Tian, Han Min, Wu Xiaohui, Xu Rener
Abstract excerpt
Dentatorubral-pallidoluysian atrophy (DRPLA) is a dominant hereditary neurodegenerative disorder caused by the expansion of a poly-glutamine (poly-Q) repeat in Atrophin-1 protein. Ectopic expression of a poly-Q expanded human Atrophin-1 is sufficient to induce DRPLA phenotypes in mice. However, it is still unclear whether the dominant effect of poly-Q expansion is due to the functional interference with wild-type...
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