Summary for peptidase S53.003: tripeptidyl-peptidase I

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

 

Names
MEROPS Nametripeptidyl-peptidase I
Other namesCLN2 g.p. (Homo sapiens), lysosomal pepstatin-insensitive proteinase, tripeptidyl aminopeptidase, tripeptidyl peptidase, TPP I, TPP1
Domain architecture
MEROPS Classification
Classification Clan SB >> Subclan (none) >> Family S53 >> Subfamily (none) >> S53.003
Holotypetripeptidyl-peptidase I (Homo sapiens), Uniprot accession O14773 (peptidase unit: 193-563), MERNUM MER0003575
History Identifier created: MEROPS 5.3 (4 December 2000)
Activity
Catalytic typeSerine
PeplistIncluded in the Peplist with identifier PL00410
NC-IUBMBSubclass 3.4 (Peptidases) >> Sub-subclass 3.4.14 (Dipeptidyl-peptidase and tripeptidyl-peptidases) >> Peptidase 3.4.14.9
EnzymologyBRENDA database
Proteolytic eventsCutDB database (6 cleavages)
Activity statushuman: active (Sohar et al., 2004)
mouse: active (Bernardini et al., 2002)
PhysiologyContributes to the lysosomal hydrolysis of hydrophobic proteins. Hereditary deficiency leads to late infantile neuronal ceroid lipofuscinosis (Rawlings & Barrett, 1999; Sohar et al., 1999). Tripeptidyl-peptidase I plays a major role in physiological degradation of cholecystokinin (Warburton & Bernardini, 2002).
KnockoutThe human disease classical late infantile neuronal ceroid lipofuscinosis (LINCL), a fatal childhood neurodegenerative disease, is due to loss-of-function mutations in tripeptidyl-peptidase I (Sleat et al., 1997; Rawlings & Barrett, 1999).
Pathways KEGGLysosome
Other databases TREEFAMhttp://www.treefam.org/family/TF333497
Cleavage site specificity Explanations of how to interpret the following cleavage site sequence logo and specificity matrix can be found here.
Cleavage pattern-/-/-/-Scissile bond-/-/-/- (based on 45 cleavages)
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Specificity matrix
 
Amino acid P4 P3 P2 P1 P1' P2' P3' P4'
Gly 0 10 4 6 5 0 3 3
Pro 1 0 9 3 1 1 2 5
Ala 0 7 3 3 2 2 2 1
Val 0 1 5 2 1 4 0 0
Leu 0 0 5 2 3 5 4 2
Ile 0 2 1 0 1 2 0 1
Met 0 0 0 6 0 2 2 0
Phe 0 3 1 7 7 4 1 3
Tyr 0 5 1 2 4 0 0 2
Trp 0 1 2 0 2 1 0 1
Ser 0 1 4 1 1 2 2 2
Thr 0 4 2 0 4 3 2 2
Cys 0 0 0 0 0 0 0 0
Asn 0 0 1 0 1 1 0 1
Gln 0 1 1 3 0 2 4 0
Asp 0 4 0 5 2 0 3 1
Glu 0 1 0 0 1 0 0 0
Lys 0 0 0 2 0 0 2 3
Arg 0 3 5 1 1 6 4 3
His 0 2 1 2 0 2 3 0
Human genetics
Gene symbol Locus Megabases Ensembl Entrez gene Gene Cards OMIM
TPP1 11p15 ENSG00000166340 1200 TPP1 204500
Mouse genetics
Gene symbol Position Megabases Ensembl Entrez gene MGI
Cln2 7:E2 ENSMUSG00000030894 12751 MGI:1336194