UniProt:P53634 CTSC

chain
  • signal peptide:1-24
  • chain:25-134
  • propeptide:135-230
  • chain:231-394
  • chain:395-463
checksum 4C9C7C24D900CEE6
comment
  • FUNCTION Thiol protease (PubMed:1586157). Has dipeptidylpeptidase activity (PubMed:1586157). Active against a broad range of dipeptide substrates composed of both polar and hydrophobic amino acids (PubMed:1586157). Proline cannot occupy the P1 position and arginine cannot occupy the P2 position of the substrate (PubMed:1586157). Can act as both an exopeptidase and endopeptidase (PubMed:1586157). Activates serine proteases such as elastase, cathepsin G and granzymes A and B (PubMed:8428921).CATALYTIC ACTIVITY Release of an N-terminal dipeptide, Xaa-Yaa-|-Zaa-, except when Xaa is Arg or Lys, or Yaa or Zaa is Pro.COFACTOR Binds 1 Cl(-) ion per heavy chain.ACTIVITY REGULATION Strongly inhibited by the cysteine peptidase inhibitors mersalyl acid, iodoacetic acid and cystatin. Inhibited by N-ethylmaleimide, Gly-Phe-diazomethane, TLCK, TPCK and, at low pH, by dithiodipyridine. Not inhibited by the serine peptidase inhibitor PMSF, the aminopeptidase inhibitor bestatin, or metal ion chelators.BIOPHYSICOCHEMICAL PROPERTIES High activity at pH 4.5-6.8.SUBUNIT Tetramer of heterotrimers consisting of exclusion domain, heavy- and light chains.INTERACTION Ubiquitous. Highly expressed in lung, kidney and placenta. Detected at intermediate levels in colon, small intestine, spleen and pancreas.INDUCTION Up-regulated in lymphocytes by IL2/interleukin-2.PTM N-glycosylated. While glycosylation at Asn-53, Asn-119 and Asn-276 is mediated by STT3A-containing complexes, glycosylation at Asn-29 is mediated STT3B-containing complexes.PTM In approximately 50% of the complexes the exclusion domain is cleaved at position 58 or 61. The two parts of the exclusion domain are held together by a disulfide bond.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the peptidase C1 family.SEQUENCE CAUTION Extended N-terminus.ONLINE INFORMATION CTSC mutation db
crossReference
databaseName UniProt
dbId 51422
description
  • recommendedName: Dipeptidyl peptidase 1 ecNumber evidence="19"3.4.14.1 alternativeName: Cathepsin C alternativeName: Cathepsin J alternativeName: Dipeptidyl peptidase I shortName: DPP-I shortName: DPPI alternativeName: Dipeptidyl transferase component recommendedName: Dipeptidyl peptidase 1 exclusion domain chain alternativeName: Dipeptidyl peptidase I exclusion domain chain /component component recommendedName: Dipeptidyl peptidase 1 heavy chain alternativeName: Dipeptidyl peptidase I heavy chain /component component recommendedName: Dipeptidyl peptidase 1 light chain alternativeName: Dipeptidyl peptidase I light chain /component
displayName UniProt:P53634 CTSC
geneName
  • CTSC
  • CPPI
identifier P53634
isSequenceChanged false
keyword
  • 3D-structure
  • Alternative splicing
  • Chloride
  • Direct protein sequencing
  • Disease variant
  • Disulfide bond
  • Glycoprotein
  • Hydrolase
  • Lysosome
  • Palmoplantar keratoderma
  • Protease
  • Proteomics identification
  • Reference proteome
  • Signal
  • Thiol protease
  • Zymogen
moleculeType Protein
name
  • CTSC
otherIdentifier
  • 1075
  • 11715710_s_at
  • 11731465_a_at
  • 11731466_a_at
  • 133_at
  • 16743111
  • 201487_PM_at
  • 201487_at
  • 225646_PM_at
  • 225646_at
  • 225647_PM_s_at
  • 225647_s_at
  • 231234_PM_at
  • 231234_at
  • 2831981
  • 3385714
  • 3385770
  • 3385771
  • 3385773
  • 3385774
  • 3385775
  • 3385776
  • 3385777
  • 3385778
  • 3385779
  • 3385780
  • 3385781
  • 3385782
  • 3385783
  • 3385786
  • 3385788
  • 3385789
  • 3385790
  • 3385791
  • 3385792
  • 3385793
  • 3385794
  • 3385795
  • 3385796
  • 3385799
  • 3385805
  • 3385807
  • 3385809
  • 3385811
  • 63866_at
  • 74213_at
  • 7893937
  • 7950906
  • A_23_P1552
  • A_24_P115762
  • A_33_P3268472
  • A_33_P3283480
  • GE61251
  • GE86110
  • GO:0001913
  • GO:0002376
  • GO:0004197
  • GO:0004252
  • GO:0005515
  • GO:0005576
  • GO:0005615
  • GO:0005654
  • GO:0005737
  • GO:0005764
  • GO:0005788
  • GO:0005813
  • GO:0005815
  • GO:0006508
  • GO:0006915
  • GO:0006955
  • GO:0008233
  • GO:0008234
  • GO:0008239
  • GO:0016020
  • GO:0016505
  • GO:0016787
  • GO:0019902
  • GO:0030134
  • GO:0030312
  • GO:0031012
  • GO:0031404
  • GO:0031410
  • GO:0031642
  • GO:0033116
  • GO:0035578
  • GO:0042802
  • GO:0051087
  • GO:0051603
  • GO:0070062
  • GO:1903052
  • GO:1903980
  • GO:2001235
  • HMNXSV003024050
  • HMNXSV003051709
  • Hs.10029.1.S1_3p_at
  • ILMN_1689086
  • ILMN_1696347
  • ILMN_1792885
  • ILMN_2242463
  • PH_hs_0022406
  • PH_hs_0035561
  • TC11002175.hg
  • X87212_at
  • g4503140_3p_at
schemaClass ReferenceGeneProduct
secondaryIdentifier
  • CATC_HUMAN
  • A8K7V2
  • B5MDD5
  • Q2HIY8
  • Q53G93
  • Q71E75
  • Q71E76
  • Q7M4N9
  • Q7Z3G7
  • Q7Z5U7
  • Q8WY99
  • Q8WYA7
  • Q8WYA8
sequenceLength 463
stId uniprot:P53634
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