Dipeptidyl peptidase 1
Also known as: CPPI, CTSC
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.
Classification
- Family (Pfam)
- PF08773 CathepsinC_exc, PF00112 Peptidase_C1
- InterPro
- CatC, CathepsinC_exc, CathepsinC_exc_dom_sf, Papain-like_cys_pep_sf, Pept_asp_AS, Pept_cys_AS, Pept_his_AS, Peptidase_C1A, Peptidase_C1A_C
- Functional cluster
- Secreted Hydrolases & Toxins
Experimental structures · PDB · 18
- 1K3B X-ray 2.15A
- 2DJF X-ray 2.00A
- 2DJG X-ray 2.05A
- 3PDF X-ray 1.85A
- 4CDC X-ray 2.40A
- 4CDD X-ray 2.35A
- 4CDE X-ray 2.40A
- 4CDF X-ray 2.20A
- 4OEL X-ray 1.40A
- 4OEM X-ray 1.52A
- 6IC5 X-ray 2.00A
- 6IC6 X-ray 1.90A
- … and 6 more
A predicted model is available from AlphaFold.
Gene Ontology · 24
- GO:0035578 azurophil granule lumen
- GO:0030134 COPII-coated ER to Golgi transport vesicle
- GO:0005788 endoplasmic reticulum lumen
- GO:0033116 endoplasmic reticulum-Golgi intermediate compartment membrane
- GO:0070062 extracellular exosome
- GO:0031012 extracellular matrix
- GO:0005576 extracellular region
- GO:0005615 extracellular space
- GO:0005764 lysosome
- GO:0016020 membrane
- GO:0031404 chloride ion binding
- GO:0004197 cysteine-type endopeptidase activity
- GO:0008234 cysteine-type peptidase activity
- GO:0008239 dipeptidyl-peptidase activity
- GO:0042802 identical protein binding
- GO:0016505 peptidase activator activity involved in apoptotic process
- GO:0019902 phosphatase binding
- GO:0051087 protein-folding chaperone binding
- GO:0004252 serine-type endopeptidase activity
- GO:0006955 immune response
- GO:1903052 positive regulation of proteolysis involved in protein catabolic process
- GO:0006508 proteolysis
- GO:0051603 proteolysis involved in protein catabolic process
- GO:0001913 T cell mediated cytotoxicity
Disease associations
- periodontitis, aggressive 1 MONDO:0008226
- Papillon-Lefevre disease MONDO:0009490
- Haim-Munk syndrome MONDO:0009491
Drugs targeting this protein · 1
- BRENSOCATIB inhibitor
Related proteins · sequence + function similarity
- Dipeptidyl peptidase 1 0.99
- Dipeptidyl peptidase 1 0.99
- Dipeptidyl peptidase 1 0.97
- Dipeptidyl peptidase 1 0.96
- Dipeptidyl peptidase 1 0.95
- Dipeptidyl peptidase 1 0.92
- Pro-cathepsin H 0.82
- Cathepsin S 0.81
- Pro-cathepsin H 0.81
- Cathepsin S 0.81
- Cathepsin S 0.80
- Cathepsin S 0.80
Co-cited proteins · studied together in the literature
- Transthyretin 1 shared papers
- Dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3B 1 shared papers
- Coagulation factor VII 1 shared papers
- Dolichyl-diphosphooligosaccharide--protein glycosyltransferase subunit STT3A 1 shared papers
- Prosaposin 1 shared papers
Literature · 31 cited papers
- N-terminome analysis of the human mitochondrial proteome. Proteomics · 2015
- Proxy molecular diagnosis from whole-exome sequencing reveals Papillon-Lefevre syndrome caused by a missense mutation in CTSC. PLoS ONE · 2015
- An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome. J. Proteomics · 2014
- Initial characterization of the human central proteome. BMC Syst. Biol. · 2011
- Cotranslational and posttranslational N-glycosylation of polypeptides by distinct mammalian OST isoforms. Cell · 2009
- Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry. J. Proteome Res. · 2009
- The full-ORF clone resource of the German cDNA consortium. BMC Genomics · 2007
- Human chromosome 11 DNA sequence and analysis including novel gene identification. Nature · 2006
- Gene symbol: CTSC. Disease: Papillon-Lefevre syndrome. Hum. Genet. · 2005
- The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. · 2004
- Loss-of-function mutations in cathepsin C in two families with Papillon-Lefevre syndrome are associated with deficiency of serine proteinases in PMNs. Hum. Mutat. · 2004
- The role of cathepsin C in Papillon-Lefevre syndrome, prepubertal periodontitis, and aggressive periodontitis. Hum. Mutat. · 2004
- … and 19 more in the literature graph
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