MOUSE MODEL FOR PREMATURE AGING: ZMPSTE24 KNOCKOUT MICE
UCLA Technology Available For Licensing

BACKGROUND:  Progerias are rare genetic diseases characterized by premature aging including: retarded growth, osteoporosis, alopecia, and ultimately occlusive vascular disease. Many progeriod disorders are caused by mutations that lead to the accumulation of a lipid-modified form of prelamin A (farnesyl-prelamin A), resulting in a disruption of the cell nucleus.

Zmpste24 is a mammalian integral membrane metalloproteinase that is critical for the processing of farnesylated proteins containing the carboxyl-terminal CAAX motif. Zmpste24, an ortholog of the yeast protein Ste24p, acts as an endoprotease by cleaving the 15 amino acids from the C terminus of prelamin A (including the farnesyl group), releasing mature lamin A.

INNOVATION:  UCLA researchers have developed Zmpste24 knockout mice in order to determine the developmental and biochemical roll of Zmpste24. Zmpste24 -/- mice have retarded growth, hair loss, muscle weakness, spontaneous bone fracture, and shortened life spans. Zmpste24 deficient cells demonstrate the accumulation of wildtype farnesyl-prelamin A along the nuclear envelope, leading to misshapen nuclei. Researchers continue to utilize the Zmpste24 knockout mice to further understand the mechanism of progerias and ultimately provide treatment options for patients.

POTENTIAL APPLICATIONS 

Related Papers (Selected)

Reference: UCLA Case No. 2007-222

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availability, please contact the following UCLA office:

UCLA Office of Intellectual Property
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Los Angeles, CA 90095-7231
Tel: 310-794-0558 Fax: 310-794-0638
email: ncd@research.ucla.edu
NCD URL:   http://www.research.ucla.edu/tech/ucla07-222.htm

Lead Inventor: Stephen Young

UCLA Technologies Available for Licensing
http://www.research.ucla.edu/oipa/industry

Copyright © 2007 The Regents of the University of California.

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