Characterisation of a transgenic ovine model of Huntington’s disease

Abstract

A transgenic ovine model of Huntington’s disease has been developed to enable the examination of the earliest disease changes in a large mammal. Ovis aries were selected because their basal ganglia and cortex is similar to analogous regions of the human brain. Importantly, they live for more than a decade, allowing for the study of the chronic effects of a fulllength HTT expressing transgene. Microinjection of a fulllength human HD cDNA containing 73 polyglutamine repeats under the control of the human promoter, resulted in six transgenic founders varying in copy-number of the transgene.