TYSND1 - trypsin like peroxisomal matrix peptidase 1 Gene

Also Known as NET41

Species: Homo sapiens

Gene Type: protein coding
Gene ID: 219743

About TYSND1

Cytogenetic location: 10q22.1 Genomic coordinates (GRCh38): 10:70,137,981-70,146,700 (from NCBI)

This gene has 4 transcripts (splice variants) and 188 orthologues. Broad expression in testis (RPKM 9.5), skin (RPKM 5.4) and 25 other tissues.

Summary

This gene encodes a protease that removes the N-terminal peroxisomal targeting signal (PTS2) from proteins produced in the cytosol, thereby facilitating their import into the peroxisome. The encoded protein is also capable of removing the C-terminal peroxisomal targeting signal (PTS1) from proteins in the peroxisomal matrix. The full-length protein undergoes self-cleavage to produce shorter, potentially inactive, peptides. Alternative splicing results in multiple transcript variants for this gene. [provided by RefSeq, Jan 2013]

TYSND1 Products (2)

mRNA Protein Name
NM_001040273.3 NP_001035363.1 peroxisomal leader peptide-processing protease isoform b
NM_173555.4 NP_775826.2 peroxisomal leader peptide-processing protease isoform a
Molecular Function GO Annotation Evidence References Source
enables identical protein binding IPI
IPI: Inferred from physical interaction
22002062 GOA
enables protease binding IPI
IPI: Inferred from physical interaction
22002062 GOA
enables protein binding IPI
IPI: Inferred from physical interaction
33961781 GOA
enables serine-type endopeptidase activity EXP
EXP: Inferred from Experiment
22002062 GOA
enables serine-type endopeptidase activity IMP
IMP: Inferred from mutant phenotype
22002062 GOA
Biological Process GO Annotation Evidence References Source
involved in protein processing IMP
IMP: Inferred from mutant phenotype
22002062 GOA
involved in proteolysis IMP
IMP: Inferred from mutant phenotype
22002062 GOA
involved in regulation of fatty acid beta-oxidation IMP
IMP: Inferred from mutant phenotype
22002062 GOA
Cellular Component GO Annotation Evidence References Source
located in peroxisome IDA
IDA: Inferred from direct assay
22002062 GOA
EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

TYSND1 Protein Structure

Trypsin_2

Trypsin_2: Trypsin-like peptidase domain (357 - 496)

  • 0
  • 100
  • 200
  • 300
  • 400
  • 500
  • 566 a.a.
Protein Preferred Names Protein Names

peroxisomal leader peptide-processing protease

  • peroxisomal cysteine endopeptidase

Related Diseases

Diseases Alias
Peroxisomal Acyl-Coa Oxidase Deficiency
  • Pseudoneonatal Adrenoleukodystrophy

  • Straight-Chain Acyl-Coa Oxidase Deficiency

  • Pseudoadrenoleukodystrophy

  • PSEUDO-NALD

  • Pseudo-Neonatal Adrenoleukodystrophy

  • Acyl-Coa Oxidase Deficiency

  • Peroxisomal Acyl-Coenzyme A Oxidase

  • Acyl-Coenzyme A Oxidase Deficiency

  • Adrenoleukodystrophy, Pseudoneonatal

  • Deficiency, Peroxisomal Acyl-Coa Oxidase

Peroxisomal Biogenesis Disorder
  • Zellweger Spectrum Disorders

  • Peroxisome Biogenesis Disorder-Zellweger Syndrome Spectrum

  • Disorders Of Peroxisome Biogenesis

  • Zellweger Spectrum

  • Zellweger Syndrome Spectrum

  • Peroxisomal Biogenesis Disorders

  • Pbd, Zss

  • Pbd-Zsd

  • Pbd-Zss

  • Pbd-Zellweger Spectrum Disorder

  • Peroxisomal Biogenesis Disorders, Zellweger Syndrome Spectrum

  • Peroxisome Biogenesis Disorder

  • Peroxisome Biogenesis Disorder Spectrum

  • Peroxisome Biogenesis Disorders

  • Zellweger Spectrum Disorder

  • Hyperpipecolic Acidaemia

Peroxisome Biogenesis Disorder 1b
  • Peroxisome Biogenesis Disorder

  • Infantile Refsum Disease

  • Infantile Phytanic Acid Storage Disease

  • PBD1B

  • Refsum Disease, Infantile

  • Adrenoleukodystrophy, Autosomal Neonatal

  • Ird

  • Mild Pbd-Zsd

  • Mild Peroxisome Biogenesis Disorder-Zellweger Spectrum Disorder

  • Pbd-Zsd

  • Peroxisome Biogenesis Disorder Spectrum

  • Peroxisome Biogenesis Disorder-Zellweger Spectrum Disorder

  • Autosomal Neonatal Adrenoleukodystrophy

  • Refsum Disease Infantile

  • Peroxisome Biogenesis Disorders

  • Peroxisome Biogenesis Disorder, Type 1b

Zellweger Syndrome
  • Cerebrohepatorenal Syndrome

  • Zellweger Leukodystrophy

  • Zs

  • Congenital Iron Overload

  • Chr

  • Zws

  • Severe Pbd-Zsd

  • Severe Peroxisome Biogenesis Disorder-Zellweger Spectrum Disorder

Diseases Alias
Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Orthologs Information

Species Symbol Source ID
Bos taurus TYSND1 VGNC VGNC:55998
Macaca mulatta TYSND1 VGNC VGNC:79722
Rattus norvegicus TYSND1 RGD RGD:1307354
Mus musculus TYSND1 MGD MGI:1919017
Others TYSND1 NCBI