CAG repeat lengths ≥335 attenuate the phenotype in the R6/2 Huntington's disease transgenic mouse

CAG repeat lengths ≥335 attenuate the phenotype in the R6/2 Huntington's disease transgenic mouse

With spontaneous elongation of the CAG repeat in the R6/2 transgene to ≥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 th...

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Bibliographic Details
Main Authors: I. Dragatsis, D. Goldowitz, N. Del Mar, Y.P. Deng, C.A. Meade, Li Liu, Z. Sun, P. Dietrich, J. Yue, A. Reiner
Format: Article
Language:English
Published: Elsevier 2009-03-01
Series:Neurobiology of Disease
Subjects:
CAG repeats
Huntington's disease
Aggregation
Nuclear entry
Pathogenesis
Striatum
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996108002568

Internet

http://www.sciencedirect.com/science/article/pii/S0969996108002568

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