Article
CAG repeat lengths > or =335 attenuate the phenotype in the R6/2 Huntington's disease transgenic mouse.
Neurobiology of disease - 1 Mar 2009
Dragatsis I, Goldowitz D, Del Mar N, Deng Y P, Meade C A, Liu Li, Sun Z, Dietrich P, Yue J, Reiner A
Abstract excerpt
With spontaneous elongation of the CAG repeat in the R6/2 transgene to > or =335, resulting in a transgene protein too large for passive entry into nuclei via the nuclear pore, we observed an abrupt increase in lifespan to >20 weeks, compared to the 12 weeks common in R6/2 mice with 150 repeats. In the > or =335 CAG mice, large ubiquitinated aggregates of mutant protein were common in neuronal dendrites and...
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