This line is transgenic for the 5' end of the human HD gene carrying &gt;(CAG)200 repeat expansions. The CAG repeat is unstable on transmission with a tendency to increase when inherited through the male line. The transgene is ubiquitously expressed. Transgenic mice exhibit a progressive neurological phenotype that mimics many of the features of HD. Onset of phenotype is apparent from approximately 8 weeks of age based on home cage behavior. However, the use of appropriate functional tests indicates the presence of a motor impairment from 5-6 weeks and cognitive impairment from 3 weeks. Epileptic seizures are seen in a small percentage of mice. A failure to gain weight is more pronounced in males than females. Immunohistochemistry with antibodies raised against the N-terminus of huntingtin reveals aggregates in the form of intranuclear inclusions and neuropil aggregates. Commonly known as the "R6/2" strain.

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This strain is discontinued, please refer to strains:<br>
<a href="https://www.jax.org/strain/002810">002810</a> B6CBA-Tg(HDexon1)62Gpb/1J<br>
<a href="https://www.jax.org/strain/006494">006494</a> B6CBA-Tg(HDexon1)62Gpb/3J

Please contact <a target="_blank" href="https://www.jax.org/jax-mice-and-services/customer-support/technical-support/contact-technical-support-form">Technical Support</a> for more information.