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Lysosomal Serine proteases that release N-terminal tripeptide from a polypeptide and cleave peptides between hydrophobic residues. An enzyme that in humans encoded by the TPP1 gene. It is involved in the degradation of bone collagen. Mutations in this gene are associated with the CEROID LIPOFUSCINOSIS, NEURONAL, 2; and SPINOCEREBELLAR ATAXIA, AUTOSOMAL RECESSIVE CEROID LIPOFUSCINOSIS, NEURONAL, 2; AND SPINOCEREBELLAR ATAXIA, AUTOSOMAL RECESSIVE 7.
Entry Term(s)
CLN2 Protein
TPP1 Protein
Tripeptidyl Aminopeptidase I
Tripeptidyl Peptidase I
Tripeptidyl Protease I
Tripeptidyl-Peptidase I
Registry Numbers
0
Related Numbers
EC 3.4.14.9
Public MeSH Note
2022; TRIPEPTIDYL-PEPTIDASES 1 was indexed under AMINOPEPTIDASES, DIPEPTIDYL-PEPTIDYL-PEPTIDASES and under SERINE PROTEASES 1995-2021
Lysosomal Serine proteases that release N-terminal tripeptide from a polypeptide and cleave peptides between hydrophobic residues. An enzyme that in humans encoded by the TPP1 gene. It is involved in the degradation of bone collagen. Mutations in this gene are associated with the CEROID LIPOFUSCINOSIS, NEURONAL, 2; and SPINOCEREBELLAR ATAXIA, AUTOSOMAL RECESSIVE CEROID LIPOFUSCINOSIS, NEURONAL, 2; AND SPINOCEREBELLAR ATAXIA, AUTOSOMAL RECESSIVE 7.