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Identification
HMDB Protein ID HMDBP08180
Secondary Accession Numbers
  • 13891
  • HMDBP09435
Name Dipeptidyl peptidase 1
Synonyms
  1. Cathepsin C
  2. Cathepsin J
  3. DPP-I
  4. DPPI
  5. Dipeptidyl peptidase 1 exclusion domain chain
  6. Dipeptidyl peptidase 1 heavy chain
  7. Dipeptidyl peptidase 1 light chain
  8. Dipeptidyl peptidase I
  9. Dipeptidyl peptidase I exclusion domain chain
  10. Dipeptidyl peptidase I heavy chain
  11. Dipeptidyl peptidase I light chain
  12. Dipeptidyl transferase
  13. SubName: Putative uncharacterized protein CTSC
Gene Name CTSC
Protein Type Unknown
Biological Properties
General Function Involved in cysteine-type endopeptidase activity
Specific 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
Pathways Not Available
Reactions Not Available
GO Classification
Function
cysteine-type peptidase activity
endopeptidase activity
cysteine-type endopeptidase activity
catalytic activity
hydrolase activity
peptidase activity
peptidase activity, acting on l-amino acid peptides
Process
metabolic process
macromolecule metabolic process
protein metabolic process
proteolysis
Cellular Location
  1. Cytoplasmic
  2. Lysosome
Gene Properties
Chromosome Location Chromosome:1
Locus 11q14.2
SNPs CTSC
Gene Sequence
>1392 bp
ATGGGTGCTGGGCCCTCCTTGCTGCTCGCCGCCCTCCTGCTGCTTCTCTCCGGCGACGGC
GCCGTGCGCTGCGACACACCTGCCAACTGCACCTATCTTGACCTGCTGGGCACCTGGGTC
TTCCAGGTGGGCTCCAGCGGTTCCCAGCGCGATGTCAACTGCTCGGTTATGGGACCACAA
GAAAAAAAAGTAGTGGTGTACCTTCAGAAGCTGGATACAGCATATGATGACCTTGGCAAT
TCTGGCCATTTCACCATCATTTACAACCAAGGCTTTGAGATTGTGTTGAATGACTACAAG
TGGTTTGCCTTTTTTAAGTATAAAGAAGAGGGCAGCAAGGTGACCACTTACTGCAACGAG
ACAATGACTGGGTGGGTGCATGATGTGTTGGGCCGGAACTGGGCTTGTTTCACCGGAAAG
AAGGTGGGAACTGCCTCTGAGAATGTGTATGTCAACACAGCACACCTTAAGAATTCTCAG
GAAAAGTATTCTAATAGGCTCTACAAGTATGATCACAACTTTGTGAAAGCTATCAATGCC
ATTCAGAAGTCTTGGACTGCAACTACATACATGGAATATGAGACTCTTACCCTGGGAGAT
ATGATTAGGAGAAGTGGTGGCCACAGTCGAAAAATCCCAAGGCCCAAACCTGCACCACTG
ACTGCTGAAATACAGCAAAAGATTTTGCATTTGCCAACATCTTGGGACTGGAGAAATGTT
CATGGTATCAATTTTGTCAGTCCTGTTCGAAACCAAGCATCCTGTGGCAGCTGCTACTCA
TTTGCTTCTATGGGTATGCTAGAAGCGAGAATCCGTATACTAACCAACAATTCTCAGACC
CCAATCCTAAGCCCTCAGGAGGTTGTGTCTTGTAGCCAGTATGCTCAAGGCTGTGAAGGC
GGCTTCCCATACCTTATTGCAGGAAAGTACGCCCAAGATTTTGGGCTGGTGGAAGAAGCT
TGCTTCCCCTACACAGGCACTGATTCTCCATGCAAAATGAAGGAAGACTGCTTTCGTTAT
TACTCCTCTGAGTACCACTATGTAGGAGGTTTCTATGGAGGCTGCAATGAAGCCCTGATG
AAGCTTGAGTTGGTCCATCATGGGCCCATGGCAGTTGCTTTTGAAGTATATGATGACTTC
CTCCACTACAAAAAGGGGATCTACCACCACACTGGTCTAAGAGACCCTTTCAACCCCTTT
GAGCTGACTAATCATGCTGTTCTGCTTGTGGGCTATGGCACTGACTCAGCCTCTGGGATG
GATTACTGGATTGTTAAAAACAGCTGGGGCACCGGCTGGGGTGAGAATGGCTACTTCCGG
ATCCGCAGAGGAACTGATGAGTGTGCAATTGAGAGCATAGCAGTGGCAGCCACACCAATT
CCTAAATTGTAG
Protein Properties
Number of Residues 463
Molecular Weight 51841.4
Theoretical pI 6.99
Pfam Domain Function
Signals
  • 1-24
Transmembrane Regions
  • None
Protein Sequence
>Dipeptidyl peptidase 1
MGAGPSLLLAALLLLLSGDGAVRCDTPANCTYLDLLGTWVFQVGSSGSQRDVNCSVMGPQ
EKKVVVYLQKLDTAYDDLGNSGHFTIIYNQGFEIVLNDYKWFAFFKYKEEGSKVTTYCNE
TMTGWVHDVLGRNWACFTGKKVGTASENVYVNTAHLKNSQEKYSNRLYKYDHNFVKAINA
IQKSWTATTYMEYETLTLGDMIRRSGGHSRKIPRPKPAPLTAEIQQKILHLPTSWDWRNV
HGINFVSPVRNQASCGSCYSFASMGMLEARIRILTNNSQTPILSPQEVVSCSQYAQGCEG
GFPYLIAGKYAQDFGLVEEACFPYTGTDSPCKMKEDCFRYYSSEYHYVGGFYGGCNEALM
KLELVHHGPMAVAFEVYDDFLHYKKGIYHHTGLRDPFNPFELTNHAVLLVGYGTDSASGM
DYWIVKNSWGTGWGENGYFRIRRGTDECAIESIAVAATPIPKL
GenBank ID Protein 189083844
UniProtKB/Swiss-Prot ID P53634
UniProtKB/Swiss-Prot Entry Name CATC_HUMAN
PDB IDs
GenBank Gene ID NM_001814.4
GeneCard ID CTSC
GenAtlas ID CTSC
HGNC ID HGNC:2528
References
General References
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  2. Ota T, Suzuki Y, Nishikawa T, Otsuki T, Sugiyama T, Irie R, Wakamatsu A, Hayashi K, Sato H, Nagai K, Kimura K, Makita H, Sekine M, Obayashi M, Nishi T, Shibahara T, Tanaka T, Ishii S, Yamamoto J, Saito K, Kawai Y, Isono Y, Nakamura Y, Nagahari K, Murakami K, Yasuda T, Iwayanagi T, Wagatsuma M, Shiratori A, Sudo H, Hosoiri T, Kaku Y, Kodaira H, Kondo H, Sugawara M, Takahashi M, Kanda K, Yokoi T, Furuya T, Kikkawa E, Omura Y, Abe K, Kamihara K, Katsuta N, Sato K, Tanikawa M, Yamazaki M, Ninomiya K, Ishibashi T, Yamashita H, Murakawa K, Fujimori K, Tanai H, Kimata M, Watanabe M, Hiraoka S, Chiba Y, Ishida S, Ono Y, Takiguchi S, Watanabe S, Yosida M, Hotuta T, Kusano J, Kanehori K, Takahashi-Fujii A, Hara H, Tanase TO, Nomura Y, Togiya S, Komai F, Hara R, Takeuchi K, Arita M, Imose N, Musashino K, Yuuki H, Oshima A, Sasaki N, Aotsuka S, Yoshikawa Y, Matsunawa H, Ichihara T, Shiohata N, Sano S, Moriya S, Momiyama H, Satoh N, Takami S, Terashima Y, Suzuki O, Nakagawa S, Senoh A, Mizoguchi H, Goto Y, Shimizu F, Wakebe H, Hishigaki H, Watanabe T, Sugiyama A, Takemoto M, Kawakami B, Yamazaki M, Watanabe K, Kumagai A, Itakura S, Fukuzumi Y, Fujimori Y, Komiyama M, Tashiro H, Tanigami A, Fujiwara T, Ono T, Yamada K, Fujii Y, Ozaki K, Hirao M, Ohmori Y, Kawabata A, Hikiji T, Kobatake N, Inagaki H, Ikema Y, Okamoto S, Okitani R, Kawakami T, Noguchi S, Itoh T, Shigeta K, Senba T, Matsumura K, Nakajima Y, Mizuno T, Morinaga M, Sasaki M, Togashi T, Oyama M, Hata H, Watanabe M, Komatsu T, Mizushima-Sugano J, Satoh T, Shirai Y, Takahashi Y, Nakagawa K, Okumura K, Nagase T, Nomura N, Kikuchi H, Masuho Y, Yamashita R, Nakai K, Yada T, Nakamura Y, Ohara O, Isogai T, Sugano S: Complete sequencing and characterization of 21,243 full-length human cDNAs. Nat Genet. 2004 Jan;36(1):40-5. Epub 2003 Dec 21. [PubMed:14702039 ]
  3. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [PubMed:15489334 ]
  4. Bechtel S, Rosenfelder H, Duda A, Schmidt CP, Ernst U, Wellenreuther R, Mehrle A, Schuster C, Bahr A, Blocker H, Heubner D, Hoerlein A, Michel G, Wedler H, Kohrer K, Ottenwalder B, Poustka A, Wiemann S, Schupp I: The full-ORF clone resource of the German cDNA Consortium. BMC Genomics. 2007 Oct 31;8:399. [PubMed:17974005 ]
  5. Taylor TD, Noguchi H, Totoki Y, Toyoda A, Kuroki Y, Dewar K, Lloyd C, Itoh T, Takeda T, Kim DW, She X, Barlow KF, Bloom T, Bruford E, Chang JL, Cuomo CA, Eichler E, FitzGerald MG, Jaffe DB, LaButti K, Nicol R, Park HS, Seaman C, Sougnez C, Yang X, Zimmer AR, Zody MC, Birren BW, Nusbaum C, Fujiyama A, Hattori M, Rogers J, Lander ES, Sakaki Y: Human chromosome 11 DNA sequence and analysis including novel gene identification. Nature. 2006 Mar 23;440(7083):497-500. [PubMed:16554811 ]
  6. Paris A, Strukelj B, Pungercar J, Renko M, Dolenc I, Turk V: Molecular cloning and sequence analysis of human preprocathepsin C. FEBS Lett. 1995 Aug 7;369(2-3):326-30. [PubMed:7649281 ]
  7. Rao NV, Rao GV, Hoidal JR: Human dipeptidyl-peptidase I. Gene characterization, localization, and expression. J Biol Chem. 1997 Apr 11;272(15):10260-5. [PubMed:9092576 ]
  8. Allende LM, Garcia-Perez MA, Moreno A, Corell A, Carasol M, Martinez-Canut P, Arnaiz-Villena A: Cathepsin C gene: First compound heterozygous patient with Papillon-Lefevre syndrome and a novel symptomless mutation. Hum Mutat. 2001 Feb;17(2):152-3. [PubMed:11180601 ]
  9. Allende LM, Moreno A, de Unamuno P: A genetic study of cathepsin C gene in two families with Papillon-Lefevre syndrome. Mol Genet Metab. 2003 Jun;79(2):146-8. [PubMed:12809647 ]
  10. Cigic B, Krizaj I, Kralj B, Turk V, Pain RH: Stoichiometry and heterogeneity of the pro-region chain in tetrameric human cathepsin C. Biochim Biophys Acta. 1998 Jan 15;1382(1):143-50. [PubMed:9507095 ]
  11. Dolenc I, Turk B, Pungercic G, Ritonja A, Turk V: Oligomeric structure and substrate induced inhibition of human cathepsin C. J Biol Chem. 1995 Sep 15;270(37):21626-31. [PubMed:7665576 ]
  12. Cigic B, Dahl SW, Pain RH: The residual pro-part of cathepsin C fulfills the criteria required for an intramolecular chaperone in folding and stabilizing the human proenzyme. Biochemistry. 2000 Oct 10;39(40):12382-90. [PubMed:11015218 ]
  13. McGuire MJ, Lipsky PE, Thiele DL: Purification and characterization of dipeptidyl peptidase I from human spleen. Arch Biochem Biophys. 1992 Jun;295(2):280-8. [PubMed:1586157 ]
  14. Turk D, Janjic V, Stern I, Podobnik M, Lamba D, Dahl SW, Lauritzen C, Pedersen J, Turk V, Turk B: Structure of human dipeptidyl peptidase I (cathepsin C): exclusion domain added to an endopeptidase framework creates the machine for activation of granular serine proteases. EMBO J. 2001 Dec 3;20(23):6570-82. [PubMed:11726493 ]
  15. Toomes C, James J, Wood AJ, Wu CL, McCormick D, Lench N, Hewitt C, Moynihan L, Roberts E, Woods CG, Markham A, Wong M, Widmer R, Ghaffar KA, Pemberton M, Hussein IR, Temtamy SA, Davies R, Read AP, Sloan P, Dixon MJ, Thakker NS: Loss-of-function mutations in the cathepsin C gene result in periodontal disease and palmoplantar keratosis. Nat Genet. 1999 Dec;23(4):421-4. [PubMed:10581027 ]
  16. Hart TC, Hart PS, Michalec MD, Zhang Y, Firatli E, Van Dyke TE, Stabholz A, Zlotogorski A, Shapira L, Soskolne WA: Haim-Munk syndrome and Papillon-Lefevre syndrome are allelic mutations in cathepsin C. J Med Genet. 2000 Feb;37(2):88-94. [PubMed:10662807 ]
  17. Hart TC, Hart PS, Michalec MD, Zhang Y, Marazita ML, Cooper M, Yassin OM, Nusier M, Walker S: Localisation of a gene for prepubertal periodontitis to chromosome 11q14 and identification of a cathepsin C gene mutation. J Med Genet. 2000 Feb;37(2):95-101. [PubMed:10662808 ]
  18. Hart PS, Zhang Y, Firatli E, Uygur C, Lotfazar M, Michalec MD, Marks JJ, Lu X, Coates BJ, Seow WK, Marshall R, Williams D, Reed JB, Wright JT, Hart TC: Identification of cathepsin C mutations in ethnically diverse papillon-Lefevre syndrome patients. J Med Genet. 2000 Dec;37(12):927-32. [PubMed:11106356 ]
  19. Nakano A, Nomura K, Nakano H, Ono Y, LaForgia S, Pulkkinen L, Hashimoto I, Uitto J: Papillon-Lefevre syndrome: mutations and polymorphisms in the cathepsin C gene. J Invest Dermatol. 2001 Feb;116(2):339-43. [PubMed:11180012 ]
  20. Lefevre C, Blanchet-Bardon C, Jobard F, Bouadjar B, Stalder JF, Cure S, Hoffmann A, Prud'Homme JF, Fischer J: Novel point mutations, deletions, and polymorphisms in the cathepsin C gene in nine families from Europe and North Africa with Papillon-Lefevre syndrome. J Invest Dermatol. 2001 Dec;117(6):1657-61. [PubMed:11886537 ]
  21. Zhang Y, Lundgren T, Renvert S, Tatakis DN, Firatli E, Uygur C, Hart PS, Gorry MC, Marks JJ, Hart TC: Evidence of a founder effect for four cathepsin C gene mutations in Papillon-Lefevre syndrome patients. J Med Genet. 2001 Feb;38(2):96-101. [PubMed:11158173 ]
  22. Zhang Y, Hart PS, Moretti AJ, Bouwsma OJ, Fisher EM, Dudlicek L, Pettenati MJ, Hart TC: Biochemical and mutational analyses of the cathepsin c gene (CTSC) in three North American families with Papillon Lefevre syndrome. Hum Mutat. 2002 Jul;20(1):75. [PubMed:12112662 ]
  23. Hewitt C, McCormick D, Linden G, Turk D, Stern I, Wallace I, Southern L, Zhang L, Howard R, Bullon P, Wong M, Widmer R, Gaffar KA, Awawdeh L, Briggs J, Yaghmai R, Jabs EW, Hoeger P, Bleck O, Rudiger SG, Petersilka G, Battino M, Brett P, Hattab F, Al-Hamed M, Sloan P, Toomes C, Dixon M, James J, Read AP, Thakker N: The role of cathepsin C in Papillon-Lefevre syndrome, prepubertal periodontitis, and aggressive periodontitis. Hum Mutat. 2004 Mar;23(3):222-8. [PubMed:14974080 ]
  24. de Haar SF, Jansen DC, Schoenmaker T, De Vree H, Everts V, Beertsen W: 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 May;23(5):524. [PubMed:15108292 ]
  25. de Haar SF, Mir M, Nguyen M, Kazemi B, Ramezani GH, Everts V, Beertsen W: Gene symbol: CTSC. Disease: Papillon-Lefevre syndrome. Hum Genet. 2005 May;116(6):545. [PubMed:15991336 ]