Article
Somatic CAG Repeat Stability in a Transgenic Sheep Model of Huntington's Disease.
Journal of Huntington's disease - 1 Jan 2024
Handley Renee R, Reid Suzanne J, Burch Zoe, Jacobsen Jessie C, Gillis Tammy, Correia Kevin, Rudiger Skye R, McLaughlin Clive J, Bawden C Simon, MacDonald Marcy E, Wheeler Vanessa C, Snell Russell G
Abstract excerpt
Somatic instability of the huntingtin (HTT) CAG repeat mutation modifies age-at-onset of Huntington's disease (HD). Understanding the mechanism and pathogenic consequences of instability may reveal therapeutic targets. Using small-pool PCR we analyzed CAG instability in the OVT73 sheep model which expresses a full-length human cDNA HTT transgene. Analyses of five- and ten-year old sheep revealed the transgene...
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