BAC-CAG transgenic mice express a neuropathogenic, full-length human mutant Huntingtin gene modified to harbor 120 CAG repeats followed by one CAA, 8 CAGs and another CAA-CAG encoding a total of 131 glutamine repeats under the control of endogenous human HTT regulatory machinery. Mice hemizygous for the BAC-CAG transgene are viable and fertile. Like other full length human HTT mouse models, BAC-CAG hemizygotes exhibit multiple phenotypes including progressive rotarod deficits (6 months), hypolocomotion (12 months), sleep disturbance (12 months), and striatal synapse loss (12 months). In addition, the BAC-CAG model, which lacks the numerous CAA interruptions in the DNA sequences encoding the mHTT polyQ stretch found in BACHD mice (FVB/N-Tg(HTT*97Q)IXwy/J, Stock No. <a href="https://www.jax.org/strain/008197">008197</a>), develops additional key Huntington's disease (HD) phenotypes such as minimal body weight gain, decrease in grip strength, striatal and cortical somatical CAG repeat instabilities correlated with behavioral impairment (locomotor activity/sleep), striatal astrocytosis and microgliosis, striatal and deeper cortical layers-selective mHTT aggregation and nuclear inclusions, and robust age-dependent and striatum-selective transcriptional dysregulation in 12-month-old mice. These mice may be useful in the testing of candidate therapeutics and in understanding the pathogenesis of Huntington's disease-like phenotypes in vivo.

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BAC-CAG transgenic mice express a neuropathogenic, full-length human mutant Huntingtin gene modified to harbor 120 CAG repeats followed by one CAA, 8 CAGs and another CAA-CAG - encoding a total of 131 glutamine repeats under the control of endogenous human HTT regulatory machinery. Hemizygous mice develop many key Huntington's disease neurodegenerative phenotypes and provide a mouse model that combines the full length human sequence (including patient-associated SNPs) with mHTT CAG repeat instability. These mice may be useful in the testing of candidate therapeutics and in understanding the pathogenesis of Huntington's disease-like phenotypes in vivo.

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