Products

Western Blot (Recombinant protein)

Enzyme-linked Immunoabsorbent Assay

Entrez GeneID

Protein Accession#

Gene Name

CTSC

Gene Alias

CPPI, DPP1, DPPI, HMS, JP, JPD, PALS, PLS

Gene Description

cathepsin C

Omim ID

Gene Ontology

Gene Summary

The protein encoded by this gene, a member of the peptidase C1 family, is a lysosomal cysteine proteinase that appears to be a central coordinator for activation of many serine proteinases in immune/inflammatory cells. It is composed of a dimer of disulfide-linked heavy and light chains, both produced from a single protein precursor, and a residual portion of the propeptide acts as an intramolecular chaperone for the folding and stabilization of the mature enzyme. This enzyme requires chloride ions for activity and can degrade glucagon. Defects in the encoded protein have been shown to be a cause of Papillon-Lefevre syndrome, an autosomal recessive disorder characterized by palmoplantar keratosis and periodontitis. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq

Other Designations

cathepsin J|dipeptidyl transferase|dipeptidyl-peptidase I