Available Technologies - Search Results
| Title: | A Reduction in Sumoylation of Cellular Proteins Reduces Polyglutamine Toxicity In Vivo in a Drosophila Fruit Fly Model of Huntington's Disease |
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| UC Case No: | 2003-114-2-ott |
| Categories: |
Pharmaceuticals:
Central Nervous System Disorders
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| Background: | Huntington's disease (HD) is a fatal, autosomal dominant, progressive neurodegenerative disorder. Clinical symptoms are progressive and include chorea, impaired voluntary movement, behavioral changes and dementia. Present therapies are limited to alleviating these symptoms without any influence on the course of the disease. HD is caused by a mutant protein, Huntingtin, which contains an expansion of a polyglutamine repeat in the amino-terminal domain of the protein. In transgenic mouse and fly models, expression of a truncated portion of the mutant Huntingtin, called Httex1p, causes disease similar to HD. |
| Technology: | University of California researchers have found that Httex1p can be modified by either SUMO-1 or ubiquitin on common lysine residues; Httex1p is stabilized by SUMO-1 modification and co-localizes with SUMO-1 in vivo and in cell culture. Crossing a Drosophila model of HD with a reduced function smt3 (Drosophila SUMO) mutant results in suppression of lethality and neurodegeneration. |
| Application: | Therefore, a drug therapy designed to lower SUMO-1 modification in the cell, or increase cleavage of SUMO-1 from target proteins, might be useful to block neurodegeneration in HD, in other polyglutamine-repeat diseases or in neurodegenerative diseases in general. |
| Contact: |
Ronnie Hanecak, Ph.D., Associate Vice Chancellor (Interim) Office of Technology Alliances University of California, Irvine 380 University Tower Irvine, CA 92697-7700 Phone: (949) 824-7186 FAX: (949) 824-2899 Email: rhanecak@uci.edu |
| Patent Status: | Pending |
| UCI School: | College of Medicine |
| Department: | Psychiatry & Human Behavior |
