Tpp1 Gene Summary [Mouse]

This gene encodes a lysosomal serine protease that cleaves N-terminal tripeptides from protein substrates. The encoded preproprotein undergoes autocatalytic processing to generate a mature enzyme. Mice lacking the encoded protein exhibit a progressive neurodegeneration and a greatly shortened lifespan. At the cellular level, mice lacking the encoded protein exhibit accumulation of autofluorescent lipopigments. Mutations in the human ortholog of this gene cause classical late-infantile neuronal ceroid lipofuscinosis. [provided by RefSeq, Nov 2015]

Details

Type
Protein Coding
Official Symbol
Tpp1
Official Name
tripeptidyl peptidase I [Source:MGI Symbol;Acc:MGI:1336194]
Ensembl ID
ENSMUSG00000030894
Bio databases IDs NCBI: 12751 Ensembl: ENSMUSG00000030894
Aliases tripeptidyl peptidase I
Synonyms CLN2, GIG1, LPIC, SCAR7, TPP-I, tripeptidyl peptidase 1, Tripeptidyl peptidase i
Species
Mouse, Mus musculus
OrthologiesHumanRat

Protein Domains

A protein domain is a distinct structural or functional region within a protein that can evolve, function, and exist independently of the rest of the protein chain. These domains in mouse Tpp1 often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
  • phospholipid binding
  • peptide binding
  • lipid binding
  • peptidase
  • protein binding
  • tripeptidyl-peptidase
  • Peptidase domain in the S8 and S53 families
  • endopeptidase
  • binding protein
  • serine-type peptidase
  • Activation domain of S53 peptidases
  • pro domain
  • glycosylation site

Pathways

Biological processes and signaling networks where the Tpp1 gene in mouse plays a role, providing insight into its function and relevance in health or disease.

Top Findings

The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
disease
  • neuronal ceroid lipofuscinosis
  • neuronal ceroid lipofuscinosis 2
  • androgenic alopecia
  • autosomal recessive spinocerebellar ataxia type 7
  • hereditary disorder
  • Angelman syndrome
  • epilepsy
  • juvenile neuronal ceroid lipofuscinosis
  • peripheral arterial disease
  • gastroesophageal reflux
regulated by
regulates
role in cell
  • cell death
  • organization
  • loss in

Subcellular Expression

Locations within the cell where the protein is known or predicted to be active, providing insight into its function and cellular context.
  • Cytoplasm
  • soluble fraction
  • lysosome
  • Golgi Apparatus
  • cytosol
  • lysosomal compartment
  • recycling endosomes
  • membrane rafts
  • telomeres
  • detergent-soluble fraction

Gene Ontology Annotations

Describes the biological processes, cellular components, and molecular functions associated with the mouse Tpp1 gene, providing context for its role in the cell.

Biological Process

Functions and activities the gene product is involved in
  • epithelial cell differentiation
  • lysosome organization
  • central nervous system development
  • peptide catabolic process
  • bone resorption
  • protein catabolic process
  • lipid metabolic process
  • proteolysis
  • protein localization to chromosome, telomeric region
  • nervous system development
  • neuromuscular process controlling balance

Cellular Component

Where in the cell the gene product is active
  • extracellular vesicular exosome
  • melanosome
  • membrane raft
  • Golgi apparatus
  • lysosomal lumen
  • recycling endosome
  • lysosome

Molecular Function

What the gene product does at the molecular level
  • protein binding
  • lysophosphatidic acid binding
  • metal ion binding
  • tripeptidyl-peptidase activity
  • serine-type peptidase activity
  • serine-type endopeptidase activity
  • endopeptidase activity
  • peptide binding
  • peptidase activity

Gene-Specific Assays for Results You Can Trust

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