AAI13851.1
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
51,854 Da
NCBI Official Full Name
CTSC protein
NCBI Official Synonym Full Names
cathepsin C
NCBI Official Synonym Symbols
JP; HMS; JPD; PLS; CPPI; DPP1; DPPI; PALS; DPP-I; PDON1 [Similar Products]
NCBI Protein Information
dipeptidyl peptidase 1; cathepsin J; dipeptidyl peptidase I; dipeptidyl transferase; dipeptidyl-peptidase I
UniProt Protein Name
Dipeptidyl peptidase 1
UniProt Synonym Protein Names
Cathepsin C; Cathepsin J; Dipeptidyl peptidase I; DPP-I; DPPI; Dipeptidyl transferaseCleaved into the following 3 chains:Dipeptidyl peptidase 1 exclusion domain chain; Alternative name(s):; Dipeptidyl peptidase I exclusion domain chainDipeptidyl peptidase 1 heavy chain; Alternative name(s):; Dipeptidyl peptidase I heavy chainDipeptidyl peptidase 1 light chain; Alternative name(s):; Dipeptidyl peptidase I light chain
UniProt Synonym Gene Names
UniProt Entry Name
CATC_HUMAN
NCBI Summary for CTSC
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, Jul 2008]
UniProt Comments for CTSC
CTSC: 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. Defects in CTSC are a cause of Papillon-Lefevre syndrome (PLS); also known as keratosis palmoplantaris with periodontopathia. PLS is an autosomal recessive disorder characterized by palmoplantar keratosis and severe periodontitis affecting deciduous and permanent dentitions and resulting in premature tooth loss. The palmoplantar keratotic phenotype vary from mild psoriasiform scaly skin to overt hyperkeratosis. Keratosis also affects other sites such as elbows and knees. Defects in CTSC are a cause of Haim-Munk syndrome (HMS); also known as keratosis palmoplantaris with periodontopathia and onychogryposis or Cochin Jewish disorder. HMS is an autosomal recessive disorder characterized by palmoplantar keratosis, onychogryphosis and periodontitis. Additional features are pes planus, arachnodactyly, and acroosteolysis. Defects in CTSC are a cause of aggressive periodontititis type 1 (AP1); also known as juvenile periodontitis (JPD) and prepubertal periodontitis (PPP). AP1 is characterized by severe and protracted gingival infections, leading to tooth loss. AP1 inheritance is autosomal dominant. Belongs to the peptidase C1 family. 3 isoforms of the human protein are produced by alternative splicing.
Protein type: EC 3.4.14.1; Endoplasmic reticulum; Protease
Chromosomal Location of Human Ortholog: 11q14.2
Cellular Component: Golgi apparatus; extracellular space; membrane; endoplasmic reticulum; lysosome
Molecular Function: identical protein binding; protein binding; protein self-association; serine-type endopeptidase activity; chaperone binding; apoptotic protease activator activity; chloride ion binding; cysteine-type peptidase activity; phosphatase binding
Biological Process: response to organic substance; apoptosis; immune response; proteolysis; T cell mediated cytotoxicity; aging
Disease: Periodontitis, Aggressive, 1; Papillon-lefevre Syndrome; Haim-munk Syndrome
Research Articles on CTSC
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Pathways associated with CTSC elisa kit
Diseases associated with CTSC elisa kit
Organs/Tissues associated with CTSC elisa kit
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