TYSND1 - trypsin like peroxisomal matrix peptidase 1 Gene

Homo sapiens

Also known as NET41

Gene ID: 219743 | Gene type: protein coding

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

Gene Ontology

Molecular Function
Biological Process
Cellular Component

Molecular Function GO Annotation	Evidence	Reference	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	Reference	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	Reference	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

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

Related Agents

Pre-clinical Phase

Bioactive Molecules (1)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-RS15311	TYSND1 Human Pre-designed siRNA Set A	Pre-designed Sets	/	Unkown

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

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