Summary for peptidase C01.070: dipeptidyl-peptidase I

Summary Gene structure Alignment Tree Sequences Sequence features Distribution Structure Literature Human EST Mouse EST Substrates Inhibitors

 

Names
MEROPS Namedipeptidyl-peptidase I
Other namescathepsin C, cathepsin J, CTH4 g.p. (Tetrahymena thermophila), CTSC g.p. (Homo sapiens), DAPase, dipeptidyl aminopeptidase I, dipeptidyl transferase, DPP I, TAGZyme
Domain architecture
MEROPS Classification
Classification Clan CA >> Subclan (none) >> Family C1 >> Subfamily A >> C01.070
Holotypedipeptidyl-peptidase I (Rattus norvegicus), Uniprot accession P80067 (peptidase unit: 229-459), MERNUM MER0000705
History Identifier created: Handbook of Proteolytic Enzymes (1998) Academic Press, London.
Activity
Catalytic typeCysteine
PeplistIncluded in the Peplist with identifier PL00068
NC-IUBMBSubclass 3.4 (Peptidases) >> Sub-subclass 3.4.14 (Dipeptidyl-peptidase and tripeptidyl-peptidases) >> Peptidase 3.4.14.1
EnzymologyBRENDA database
Activity statushuman: active (Turk et al., 2004)
mouse: active (Adkison et al., 2002)
PhysiologyA function is the activation of granulocyte serine endopeptidases by removal of N-terminal dipeptides (e.g. Pham et al., 1999).
KnockoutMutations in the gene for dipeptidyl-peptidase I lead to Papillon-Lefevre syndrome, an autosomal recessive disorder characterised by palmoplantar keratoderma and severe, early onset periodontitis (Hart et al., 1999; Toomes et al., 1999; Hart et al., 2000). Haim-Munk syndrome is also caused by mutations in dipeptidyl-peptidase I (Cury et al., 2005).
Pharmaceutical relevanceMice lacking dipeptidyl-peptidase I showed improved survival of sepsis, suggesting that the peptidase may be a therapeutic target for treatment of sepsis (Mallen-St Clair et al., 2004).
Pathways KEGGLysosome
Other databases WIKIPEDIAhttp://en.wikipedia.org/wiki/Cathepsin_C
Cleavage site specificity Explanations of how to interpret the following cleavage site sequence logo and specificity matrix can be found here.
Cleavage pattern-/s/-/esScissile bond-/-/-/gr (based on 33 cleavages)
weblogo
Specificity matrix
 
Amino acid P4 P3 P2 P1 P1' P2' P3' P4'
Gly 2 2 4 3 2 2 7 7
Pro 0 0 0 0 0 1 0 0
Ala 1 4 2 0 1 0 2 0
Val 1 0 0 0 0 1 0 0
Leu 2 2 2 2 2 3 4 3
Ile 0 0 0 0 8 6 0 0
Met 0 0 0 0 1 0 0 0
Phe 0 2 1 3 0 3 1 2
Tyr 1 1 2 1 1 2 2 2
Trp 0 0 0 1 0 0 0 0
Ser 2 5 3 7 2 4 3 4
Thr 4 0 4 0 4 0 4 0
Cys 0 1 0 0 0 0 0 0
Asn 0 0 1 0 0 0 0 1
Gln 1 0 2 1 2 2 1 3
Asp 4 0 5 0 4 1 3 0
Glu 1 0 5 8 1 1 1 0
Lys 0 1 0 2 1 1 1 2
Arg 0 3 0 5 2 4 2 5
His 2 0 2 0 0 0 0 0
Human genetics
Gene symbol Locus Megabases Ensembl Entrez gene Gene Cards OMIM
CTSC 11q14.1-q14.3 ENSG00000109861 1075 CTSC 602365
Mouse genetics
Gene symbol Position Megabases Ensembl Entrez gene MGI
Ctsc 7:E1 ENSMUSG00000030560 13032 MGI:109553