These mice manifest the same defects seen in people afflicted by autosomal recessive ichthyosis with hypotricosis so it can be an effective model for studying the role of matriptase in disorders that affect skin development.

Available for licensing are mice with greatly reduced levels of matriptase, a membrane protease involved in epithelial development, immune function, and carcinogenesis. These mice were created to study autosomal recessive ichthyosis with hypotricosis (ARIH), an inherited human disease that has been linked to a mutation in the ST14 gene that encodes matriptase.  These mice manifest the same defects seen in people afflicted by ARIH so it can be an effective model for studying the role of matriptase in disorders that affect skin development.

 

Applications:

Research tool for skin development research

Model to develop and test therapeutics for treating skin disorders, including skin cancer

Model immunity and allergy

 

Advantages:

Well characterized animal model closely related to a human genetic disorder.

 

Market:

Ichthyosis is a series of genetic skin diseases characterized by dry, thickened, scaling skin that affects more than one million Americans. Presently, there is no cure for ichthyosis, only treatments to help manage symptoms.

 

Development Status:

Well characterized mouse model of human ARIH.

 

Publication:

K List et al. Autosomal ichthyosis with hypotrichosis syndrome displays low matriptase proteolytic activity and is phenocopied in ST14 hypomorphic mice. J Biol Chem. 2007 Dec 14;282(50):36714-36723.  [PubMed abs]

 

 

HHS Reference No. E-323-2008/0 – Biological Material.  Patent protection is not being pursued for this technology.

 

Inventors:

Thomas H. Bugge (NIDCR) et al.

 

 

Licensees Sought:

Available for non-exclusive licensing under a Biological Materials License Agreement.

 

Collaborative Research Opportunity:

The National Institute of Dental and Craniofacial Research, Oral and Pharyngeal Cancer Branch, is seeking statements of capability or interest from parties interested in collaborative research to further develop, evaluate, or commercialize this technology.  Please contact David W. Bradley, Ph.D. at 301-402-0540 or bradleyda@nidcr.nih.gov for more information.