Cre/LoxP Conditional Mouse Model of Huntington's Disease
|
UCLA Technology Available For Licensing |
BACKGROUND:
Huntington's disease (HD) is an adult-onset, autosomal-dominant neurodegenerative disease that is clinically characterized by a triad of movement disorders (i.e., chorea and bradykinesia), psychiatric symptoms, and cognitive deficits. In afflicted patients, symptoms usually progress relentlessly until death in 15-20 years after disease onset. HD is one of nine neurodegenerative disorders caused by a CAG repeat expansion encoding a polyglutamine (polyQ) repeat in otherwise unrelated proteins. In HD, the mutated Huntingtin (mhtt) protein is ubiquitously expressed in both neuronal and nonneuronal tissues. The polyQ repeat, located in the N terminus of huntingtin (htt), is normally less than 36, but is expanded to more than 37 in HD patients. In all polyQ disorders, there is an inverse relationship between the length of polyQ and the age of disease onset. Currently, there is no effective treatment or cure for HD or any other polyQ disorder.
INNOVATION:
Researchers at UCLA have developed a Cre/LoxP conditional mouse model of HD (termed RosaHD mice) in which expression of a toxic mutant huntingtin Exon 1 (mhtt-exon1) fragment, driven by the endogenous ubiquitously-expressing Rosa26 promoter, can be switched on by Cre recombinase.
POTENTIAL APPLICATIONS
- Useful in studying Huntington's disease (HD) or other polyQ disorders.
Related Papers (Selected)
- Pathological cell-cell interactions elicited by a neuropathogenic form of mutant Huntingtin contribute to cortical pathogenesis in HD mice [more]
- Pathological cell-cell interactions are necessary for striatal pathogenesis in a conditional mouse model of Huntington's disease [more]
- Information on Mouse Strain from The Jackson Laboratory [more]
Reference: UCLA Case No. 2009-522
For additional technical details and current licensing
availability,
please contact the following UCLA office:
UCLA Office of Intellectual Property
11000 Kinross Avenue, Suite #200
Los Angeles, CA 90095
Tel: 310-794-0558 Fax: 310-794-0638
email: ncd@research.ucla.edu
|
NCD URL: http://www.research.ucla.edu/tech/ucla09-522.htm
|
UCLA Technologies Available for
Licensing
http://www.research.ucla.edu/oipa/industry
Copyright © 2009 The Regents of the
University of California.
keywords: research tools, Huntington's disease, polyQ disorder, mouse model, Huntingtin protein
uclancd ucla latest inventions technology top ten 10 technologies intellectual property patents technology transfer invention business card