Article
A critical window of CAG repeat-length correlates with phenotype severity in the R6/2 mouse model of Huntington's disease.
Journal of neurophysiology - 1 Jan 2012
Cummings Damian M, Alaghband Yasaman, Hickey Miriam A, Joshi Prasad R, Hong S Candice, Zhu Chunni, Ando Timothy K, André Véronique M, Cepeda Carlos, Watson Joseph B, Levine Michael S
Abstract excerpt
The R6/2 mouse is the most frequently used model for experimental and preclinical drug trials in Huntington's disease (HD). When the R6/2 mouse was first developed, it carried exon 1 of the huntingtin gene with ~150 cytosine-adenine-guanine (CAG) repeats. The model presented with a rapid and aggressive phenotype that shared many features with the human condition and was particularly similar to juvenile HD....
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