Article
Ectopically expressed CAG repeats cause intranuclear inclusions and a progressive late onset neurological phenotype in the mouse.
Cell - 12 Dec 1997
Ordway J M, Tallaksen-Greene S, Gutekunst C A, Bernstein E M, Cearley J A, Wiener H W, Dure L S, Lindsey R, Hersch S M, Jope R S, Albin R L, Detloff P J
Abstract excerpt
The mutations responsible for several human neurodegenerative disorders are expansions of translated CAG repeats beyond a normal size range. To address the role of repeat context, we have introduced a 146-unit CAG repeat into the mouse hypoxanthine phosphoribosyltransferase gene (Hprt). Mutant mice express a form of the HPRT protein that contains a long polyglutamine repeat. These mice develop a phenotype similar...
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