NIH grant establishes Mouse Mutant Peroxisome Research Resource

Aamir Zuberi (center) leads a tour with Mary Jo Kutler and Teri Koerner with the Snyder Robinson Foundation. September 2019. Photo credit: Tiffany LauferAamir Zuberi (center) leads a tour with Mary Jo Kutler and Teri Koerner with the Snyder Robinson Foundation. Photo credit: Tiffany Laufer

Jackson Laboratory Director, Technology Evaluation and Development Aamir Zuberi, Ph.D., along with co-principal investigators Joseph Hacia, Ph.D., of the University of Southern California and Nancy Braverman, M.D., from the McGill University Health Center in Montreal, Canada, have received a four-year, $3 million NIH grant to establish a Mouse Mutant Peroxisome Research Resource at The Jackson Laboratory. 

Peroxisomes are metabolic organelles that act as a central hub of cell signaling pathways and play essential roles in the function and development of all organ systems. More than 70 proteins have been reported to localize to the peroxisome matrix and 20 to the peroxisome membrane, and peroxisome dysfunction is associated with numerous common disorders as well as aging. Peroxisome biogenesis disorders (PBDs) are rare recessive disorders caused by defects in any of 14 PEX genes encoding the peroxin proteins necessary for proper peroxisome assembly. PBD patients lack sufficient preclinical resources to facilitate an understanding of disease progression, and the lack of preclinical mouse models limit options in developing new therapeutic options for treatment. 

Supported by the grant, Zuberi and his team will facilitate the generation, characterization and distribution of new peroxisome mouse models and generate new monoclonal antibodies targeted to specific peroxisomal proteins.

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