Rabbit anti-Human Cleaved-CTSC (R394) Polyclonal Antibody

Uniprot Id

P53634

Target Species

Human

Target Name

CTSC

Target Full Name

Dipeptidyl peptidase 1

Target Function

Thiol protease. Has dipeptidylpeptidase activity. Active against a broad range of dipeptide substrates composed of both polar and hydrophobic amino acids. Proline cannot occupy the P1 position and arginine cannot occupy the P2 position of the substrate. Can act as both an exopeptidase and endopeptidase. Activates serine proteases such as elastase, cathepsin G and granzymes A and B. Can also activate neuraminidase and factor XIII.

Target Involvement

Papillon-Lefevre syndrome (PLS); Haim-Munk syndrome (HMS); Periodontititis, aggressive, 1 (AP1)

Target Subcellular Location

Lysosome.

Target Protein Families

Peptidase C1 family

Target Tissue Specificity

Ubiquitous. Highly expressed in lung, kidney and placenta. Detected at intermediate levels in colon, small intestine, spleen and pancreas.

Target Synonyms

AI047818; CATC; CATC_HUMAN; Cathepsin C; Cathepsin J; CPPI; CTSC; Dipeptidyl peptidase 1; Dipeptidyl peptidase 1 light chain; Dipeptidyl peptidase I; Dipeptidyl peptidase I exclusion domain chain; Dipeptidyl peptidase I heavy chain; Dipeptidyl peptidase I light chain; Dipeptidyl transferase; DPP I; DPP-I; DPPI; EC 3.4.14.1; HMS; JP; JPD; MGC126959 ; PALS; PDON1; PLS

Target Background

This gene encodes a member of the peptidase C1 family and lysosomal cysteine proteinase that appears to be a central coordinator for activation of many serine proteinases in cells of the immune system. Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed to generate heavy and light chains that form a disulfide-linked dimer. A 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.