Chemicals and Drugs

Tripeptidyl-Peptidase 1

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.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

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.

MeSH Record

Classification

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MeSH Record

Synonyms

9 entry terms
  • CLN2 Protein
  • TPP1 Protein
  • Tripeptidyl Aminopeptidase I
  • Tripeptidyl Peptidase I
  • Tripeptidyl Protease I
  • Tripeptidyl-Peptidase I
  • Peptidase I, Tripeptidyl
  • Protein, CLN2
  • Protein, TPP1

MeSH Record

Aspects Covered

29 allowable subheadings

Indexed with the subheadings administration & dosage, adverse effects, analysis, antagonists & inhibitors, biosynthesis, blood, cerebrospinal fluid, chemical synthesis, chemistry, classification, deficiency, drug effects, economics, genetics, history, immunology, isolation & purification, metabolism, pharmacokinetics, pharmacology, physiology, poisoning, radiation effects, standards, supply & distribution, therapeutic use, toxicity, ultrastructure, urine.

MeSH Record

History Note

2022 (1993)

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AMA Style

References

  1. National Library of Medicine. Tripeptidyl-Peptidase 1. Medical Subject Headings (MeSH). 2026. Unique ID D000091346. http://id.nlm.nih.gov/mesh/2026/D000091346
  2. Tripeptidyl-Peptidase 1. In: Wikidata. https://www.wikidata.org/wiki/Q21136439