Characterization of mouse Clpp protease cDNA, gene, and protein

Mutations that cause accumulation or rapid degradation owing to protein mis folding are a frequent cause of inherited disease in humans. In Escherichia coli, Clpp protease is one of the components of the protein quality contro l system that handles misfolded proteins. In the present study, we have cha racterized the mouse Clpp cDNA sequence, the organization of the mouse gene , the chromosomal localization, and the tissue-specific expression pattern. Moreover, the cellular localization and processing of mouse Clpp was studi ed by overexpression in transfected eukaryotic cells. Our results indicate that mouse and human Clpp have similar roles, and they provide the molecula r basis for establishing a Clpp knockout mouse and to study its phenotype, thereby shedding light on a possible role of Clpp in human disease.