$VAR1 = undef;

Summary for peptidase S53.003: tripeptidyl-peptidase I

Names
MEROPS Name	tripeptidyl-peptidase I
Other names	Cerliponase alfa, CLN2 g.p. (Homo sapiens), lysosomal pepstatin-insensitive proteinase, tripeptidyl aminopeptidase, tripeptidyl peptidase, tripeptidyl peptidase 1, TPP I, TPP1

Domain architecture

MEROPS Classification
Classification	Clan SB >> Subclan (none) >> Family S53 >> Subfamily (none) >> S53.003
Holotype	tripeptidyl-peptidase I (Homo sapiens), Uniprot accession O14773 (peptidase unit: 193-563), MERNUM MER0003575
History	Identifier created: MEROPS 5.3 (4 December 2000)

Activity
Catalytic type	Serine
Peplist	Included in the Peplist with identifier PL00410
NC-IUBMB	Subclass 3.4 (Peptidases) >> Sub-subclass 3.4.14 (Dipeptidyl-peptidase and tripeptidyl-peptidases) >> Peptidase 3.4.14.9
Enzymology	BRENDA database
Proteolytic events	CutDB database (6 cleavages)
Activity status	human: active (Sohar et al., 2004) mouse: active (Bernardini et al., 2002)
Physiology	Contributes 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).
Knockout	The 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	KEGG	Lysosome
Other databases	TREEFAM	http://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	-/-/-/--/-/-/- (based on 45 cleavages)

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