Seroatlas · Human Serome Atlas

TPP1

Tripeptidyl-peptidase 1

Also known as: CLN2, LPIC, SCAR7, TPP-1, TPP1_HUMAN

Cross-references: UniProt · Ensembl · Human Protein Atlas · GeneCards · NCBI Gene

Protein identityUniProt · HPA

UniProt accession
O14773
Gene
TPP1
Ensembl
ENSG00000166340
Chromosome
11
Canonical length
563 aa
Protein class
Disease related genes, Enzymes, Human disease related genes, Metabolic proteins, Plasma proteins, Potential drug targets, Predicted intracellular proteins
Secretome location
Intracellular and membrane

OverviewNCBI Gene

This gene encodes a member of the sedolisin family of serine proteases. The protease functions in the lysosome to cleave N-terminal tripeptides from substrates, and has weaker endopeptidase activity. It is synthesized as a catalytically-inactive enzyme which is activated and auto-proteolyzed upon acidification. Mutations in this gene result in late-infantile neuronal ceroid lipofuscinosis, which is associated with the failure to degrade specific neuropeptides and a subunit of ATP synthase in the lysosome. [provided by RefSeq, Jul 2008]

Canonical amino-acid sequenceUniProt

563 residues, UniProt reviewed canonical sequence.

>O14773|TPP1
     1  MGLQACLLGL FALILSGKCS YSPEPDQRRT LPPGWVSLGR ADPEEELSLT FALRQQNVER
    61  LSELVQAVSD PSSPQYGKYL TLENVADLVR PSPLTLHTVQ KWLLAAGAQK CHSVITQDFL
   121  TCWLSIRQAE LLLPGAEFHH YVGGPTETHV VRSPHPYQLP QALAPHVDFV GGLHRFPPTS
   181  SLRQRPEPQV TGTVGLHLGV TPSVIRKRYN LTSQDVGSGT SNNSQACAQF LEQYFHDSDL
   241  AQFMRLFGGN FAHQASVARV VGQQGRGRAG IEASLDVQYL MSAGANISTW VYSSPGRHEG
   301  QEPFLQWLML LSNESALPHV HTVSYGDDED SLSSAYIQRV NTELMKAAAR GLTLLFASGD
   361  SGAGCWSVSG RHQFRPTFPA SSPYVTTVGG TSFQEPFLIT NEIVDYISGG GFSNVFPRPS
   421  YQEEAVTKFL SSSPHLPPSS YFNASGRAYP DVAALSDGYW VVSNRVPIPW VSGTSASTPV
   481  FGGILSLINE HRILSGRPPL GFLNPRLYQQ HGAGLFDVTR GCHESCLDEE VEGQGFCSGP
   541  GWDPVTGWGT PNFPALLKTL LNP

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against TPP1 can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.

Antibody reachability
Intracellular
Secreted
No
Transmembrane segments
0
Mean surface accessibility (rSASA)
0.22
Highest tissue expression
172 nTPM

Expression across tissuesHPA

Tissue

  • choroid plexus: 172 nTPM
  • spleen: 122 nTPM
  • bone marrow: 107 nTPM
  • placenta: 89 nTPM
  • liver: 89 nTPM
  • smooth muscle: 86 nTPM

Single-cell type

  • adrenal cortex cells: 96 nCPM
  • astrocytes: 40 nCPM
  • choroid plexus epithelial cells: 37 nCPM
  • platelets: 35 nCPM
  • microglia: 32 nCPM
  • monocytes: 30 nCPM

Immune cell

  • myeloid DC: 187 nTPM
  • intermediate monocyte: 172 nTPM
  • classical monocyte: 168 nTPM
  • total PBMC: 140 nTPM
  • non-classical monocyte: 127 nTPM
  • plasmacytoid DC: 93 nTPM

Brain region

  • choroid plexus: 197 nTPM
  • white matter: 134 nTPM
  • medulla oblongata: 126 nTPM
  • thalamus: 123 nTPM
  • midbrain: 118 nTPM
  • cerebellum: 112 nTPM

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about TPP1.

Disease | AllUniProt

Conditions TPP1 is implicated in, by any mechanism.

  • Ceroid lipofuscinosis, neuronal, 2 (CLN2) MIM:204500
  • Spinocerebellar ataxia, autosomal recessive, 7 (SCAR7) MIM:609270

Disease | GeneticClinVar

221 pathogenic / likely-pathogenic of 1,289 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

Genetic constraint and essentialitygnomAD · DepMap

Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.

gnomAD LOEUF (loss-of-function intolerance)
0.78
gnomAD pLI
0
gnomAD missense Z
-0.22
DepMap mean gene effect
0.02
DepMap dependency class
none

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 4% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).

OntologyGO

Biological processes

Molecular functions

Cellular components

Protein domainsUniProt · Pfam · InterPro

KeywordsUniProt

InteractionsUniProt · HPA

Protein binding partners of TPP1 in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

Antibody and autoantibody relevanceSeroatlas analysis

Seroatlas reads TPP1 as an antibody target. Whether an autoantibody or antibody against TPP1 could matter depends on whether native TPP1 is physically reachable, whether the body needs it intact, and whether it acts in a disease-relevant tissue.

TPP1 is annotated as predominantly intracellular. Intracellular proteins are common autoantibody markers, becoming visible to the immune system after cell injury or altered processing, but are usually markers of disease rather than direct drivers.

Annotation status

The present source text does not explicitly label TPP1 as an autoantigen. Seroatlas presents hypothesis context only and does not manufacture a known-serology claim.

Canonical record: https://seroatlas.com/gene/TPP1. Study-independent annotations aggregated from UniProt, Human Protein Atlas, PubMed, IEDB, Pfam, InterPro, Gene Ontology, AlphaFold, gnomAD, DepMap, ClinVar, TCGA. Catalog release seroatlas-reviewed-human-uniprot-20260313.

Seroatlas is the reference for exploring autoantibody and antibody serology at the human-protein level: the autoreactome and human serome, multiplex serology (HuProt, HuScan, VirScan, PhIP-Seq).

© 2026 · Seroatlas, Discover Oregon LLC · Terms of use

Loading the interactive Seroatlas protein explorer...