HTRA2 - HtrA serine peptidase 2 Gene

Homo sapiens

Also known as OMI; MGCA8; PARK13; PRSS25

Gene ID: 27429 | Gene type: protein coding

About HTRA2

Cytogenetic location: 2p13.1 Genomic coordinates (GRCh38): 2:74,529,405-74,533,556 (from NCBI)

This gene has 17 transcripts (splice variants), 195 orthologues, 3 paralogues and is associated with 5 phenotypes. Ubiquitous expression in spleen (RPKM 12.3), bone marrow (RPKM 11.8) and 25 other tissues.

Summary

This gene encodes a serine protease. The protein has been localized in the endoplasmic reticulum and interacts with an alternatively spliced form of mitogen-activated protein kinase 14. The protein has also been localized to the mitochondria with release to the cytosol following apoptotic stimulus. The protein is thought to induce Apoptosis by binding the Apoptosis inhibitory protein baculoviral IAP repeat-containing 4. Nuclear localization of this protein has also been observed. Alternate splicing of this gene results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Mar 2016]

HTRA2 Products(4)

mRNA	Protein	Name
NM_001321727.1	NP_001308656.1	serine protease HTRA2, mitochondrial isoform 3 preproprotein
NM_001321728.1	NP_001308657.1	serine protease HTRA2, mitochondrial isoform 4 preproprotein
NM_013247.5	NP_037379.1	serine protease HTRA2, mitochondrial isoform 1 preproprotein
NM_145074.2	NP_659540.1	serine protease HTRA2, mitochondrial isoform 2 preproprotein

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	11967569	GOA
enables peptidase activity	IDA IDA: Inferred from direct assay	11604410	GOA
enables peptidase activity	IMP IMP: Inferred from mutant phenotype	24709290	GOA
enables protein binding	IPI IPI: Inferred from physical interaction	10971580	GOA
enables protein serine/threonine kinase inhibitor activity	IDA IDA: Inferred from direct assay	25118933	GOA
enables serine-type endopeptidase activity	IDA IDA: Inferred from direct assay	11602612	GOA
enables serine-type endopeptidase activity	IMP IMP: Inferred from mutant phenotype	10971580	GOA
enables serine-type peptidase activity	IDA IDA: Inferred from direct assay	11583623	GOA
enables ubiquitin ligase inhibitor activity	IDA IDA: Inferred from direct assay	11602612	GOA

Biological Process GO Annotation	Evidence	Reference	Source
involved in cellular response to growth factor stimulus	IMP IMP: Inferred from mutant phenotype	20125124	GOA
involved in cellular response to heat	IDA IDA: Inferred from direct assay	10971580	GOA
involved in cellular response to interferon-beta	IDA IDA: Inferred from direct assay	17297443	GOA
involved in cellular response to oxidative stress	IMP IMP: Inferred from mutant phenotype	24709290	GOA
involved in cellular response to retinoic acid	IDA IDA: Inferred from direct assay	17297443	GOA
involved in intracellular signal transduction	IDA IDA: Inferred from direct assay	25118933	GOA
involved in intrinsic apoptotic signaling pathway in response to DNA damage	IMP IMP: Inferred from mutant phenotype	11602612	GOA
involved in mitochondrion organization	IMP IMP: Inferred from mutant phenotype	25118933	GOA
involved in positive regulation of apoptotic process	IMP IMP: Inferred from mutant phenotype	11967569	GOA
involved in positive regulation of cysteine-type endopeptidase activity involved in apoptotic process	IDA IDA: Inferred from direct assay	11583623	GOA
involved in positive regulation of cysteine-type endopeptidase activity involved in apoptotic process	IMP IMP: Inferred from mutant phenotype	11967569	GOA
involved in positive regulation of execution phase of apoptosis	IDA IDA: Inferred from direct assay	17297443	GOA
involved in positive regulation of extrinsic apoptotic signaling pathway in absence of ligand	IMP IMP: Inferred from mutant phenotype	20125124	GOA
involved in programmed cell death	IDA IDA: Inferred from direct assay	11583623	GOA
involved in protein catabolic process	IDA IDA: Inferred from direct assay	17297443	GOA
involved in proteolysis	IMP IMP: Inferred from mutant phenotype	10971580	GOA

Cellular Component GO Annotation	Evidence	Reference	Source
located in chromatin	IDA IDA: Inferred from direct assay	24798695	GOA
located in cytoskeleton	IDA IDA: Inferred from direct assay	24798695	GOA
located in cytosol	IDA IDA: Inferred from direct assay	11583623	GOA
located in membrane	IDA IDA: Inferred from direct assay	24798695	GOA
located in mitochondrial intermembrane space	IDA IDA: Inferred from direct assay	15044455	GOA
located in mitochondrion	IDA IDA: Inferred from direct assay	11583623	GOA
located in mitochondrion	IMP IMP: Inferred from mutant phenotype	11604410	GOA
located in nucleus	IDA IDA: Inferred from direct assay	10971580	GOA
part of serine-type endopeptidase complex	IMP IMP: Inferred from mutant phenotype	11967569	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

HTRA2 Protein Structure

Trypsin_2

PDZ_2

0
100
200
300
400
458 a.a.

Protein Preferred Names

Protein Names

serine protease HTRA2, mitochondrial

HtrA-like serine protease

HTRA2 Protein-protein interaction Information

Type Cross Intra	Protein Name	Protein ID	Interactor	Interactor Species	Interactor ID	Detection Method	Reference
Intra	HTRA2	O43464	PSMC3	Homo sapiens	P17980	Validated Y2H	32814053
Intra	HTRA2	O43464	PSMC3	Homo sapiens	P17980	Y2H Array	32814053
Intra	HTRA2	O43464	PSMC3	Homo sapiens	P17980	Y2H Pooling	32814053
Intra	HTRA2	O43464	NDUFA13	Homo sapiens	Q9P0J0	BioID	31617661
Intra	HTRA2	O43464	NDUFA13	Homo sapiens	Q9P0J0	Anti Bait CoIP	17297443
Intra	HTRA2	O43464	NDUFA13	Homo sapiens	Q9P0J0	Anti Tag CoIP	17297443
Intra	HTRA2	O43464	NDUFA13	Homo sapiens	Q9P0J0	Y2H	17297443
Intra	HTRA2	O43464	SMN1	Homo sapiens	Q16637-3	Validated Y2H	32814053
Intra	HTRA2	O43464	SMN1	Homo sapiens	Q16637-3	Y2H Array	32814053
Intra	HTRA2	O43464	SMN1	Homo sapiens	Q16637-3	Y2H Pooling	32814053
Intra	HTRA2	O43464	BIRC2	Homo sapiens	Q13490	BioID	31617661
Intra	HTRA2	O43464	XIAP	Homo sapiens	P98170	SPR	24698088
Intra	HTRA2	O43464	XIAP	Homo sapiens	P98170	BioID	31617661
Intra	HTRA2	O43464	XIAP	Homo sapiens	P98170	Protease Assay	28642151
Intra	HTRA2	O43464	XIAP	Homo sapiens	P98170	FPS	11801603
Intra	HTRA2	O43464	XIAP	Homo sapiens	P98170	Pull Down	11583623
Intra	HTRA2	O43464	XIAP	Homo sapiens	P98170	Pull Down	11801603
Intra	HTRA2	O43464	XIAP	Homo sapiens	P98170	Anti Tag CoIP	11604410
Intra	HTRA2	O43464	XIAP	Homo sapiens	P98170	Pull Down	24698088
Intra	HTRA2	O43464	XIAP	Homo sapiens	P98170	CoIP	11602612
Cross	HTRA2	O43464	Ripk1	Mus musculus	Q60855	Protease Assay	20125124
Intra	HTRA2	O43464	TTC19	Homo sapiens	Q6DKK2	BioID	31617661
Cross	HTRA2	O43464	CSN2	Bos taurus	P02666	Protease Assay	28642151
Cross	HTRA2	O43464	CSN2	Bos taurus	P02666	Protease Assay	10971580

Cross: Cross-species interaction Intra: Intraspecies interaction

Recombinant HTRA2 Proteins

Cat. No.	Product Name	Accession	Purity
HY-P74876	HTRA2/OMI Protein, Human (His)	O43464 (A134-E458)	≥95%

Related Diseases

Diseases

Alias

3-Methylglutaconic Aciduria, Type Viii

3-Methylglutaconic Aciduria Type 8	MGCA8
3-Methylglutaconic Aciduria Type Viii, Mgca8	Mga8
3-Methylglutaconic Aciduria 8	3-Methylglutaconic Aciduria, Type Vii

Parkinson Disease 13, Autosomal Dominant

Parkinson Disease 13	Parkinson Disease 13, Autosomal Dominant, Susceptibility To
PARK13	Parkinson Disease, Type 13

3-Methylglutaconic Aciduria

3-Methyl Glutaconic Aciduria

Parkinson Disease 2, Autosomal Recessive Juvenile

Young-Onset Parkinson Disease	PARK2
Pdj	Autosomal Recessive Juvenile Parkinson Disease 2
Epdf	Parkinson Disease, Juvenile, Type 2
Parkinson'S Disease 2	Autosomal Recessive Juvenile Parkinson Disease
Early-Onset Parkinson Disease	Parkinson Disease 2
Parkinson Disease, Juvenile, Autosomal Recessive	Parkinsonism, Early-Onset, With Diurnal Fluctuation
Autosomal Recessive Juvenile Parkinson'S Disease 2	Jp
Juvenile Parkinsonism	Parkinson Disease Autosomal Recessive, Early Onset
Parkinsonism, Early Onset, With Diurnal Fluctuation	Yopd
Autosomal Recessive Early-Onset Parkinson Disease Type 2	Chromosome 6-Linked Autosomal Recessive Parkinsonism
Early-Onset Parkinsonism With Diurnal Fluctuation	Parkinsonism Young Adult Onset
Parkinson Disease, Type 2	Parkinsonism, Juvenile

Tremor

Medicament-Induced Tremor

Medication-Induced Postural Tremor

Essential Tremor

Benign Essential Tremor	Familial Tremor
Hereditary Essential Tremor	Essential Hereditary Tremor
Shaky Hand Syndrome	Benign Essential Tremor Syndrome
Tremor Hereditary Essential	Essential Tremor, Susceptibility To
Tremor, Hereditary Essential

Tremor, Hereditary Essential, 1

ETM1	Fet1
Essential Tremor 1	Tremor, Familial Essential, 1
Essential Tremor, Hereditary, 1	Hereditary Essential Tremor 1
Tremor Hereditary Essential, 1	Tremor Familial Essential, 1
Tremor, Hereditary Essential 1	Tremor, Essential Hereditary, Type 1

Parkinson Disease 3, Autosomal Dominant

PARK3	Parkinson Disease 3
Parkinson Disease 3, Autosomal Dominant Lewy Body	Parkinson'S Disease 3
Autosomal Dominant Lewy Body Parkinson Disease 3	Autosomal Dominant Parkinson Disease 3
Parkinson Disease Type 3	Autosomal Dominant Parkinson Disease
Parkinson Disease, Autosomal Dominant	Parkinson Disease, Familial, Type 1

3-Methylglutaconic Aciduria With Cataracts, Neurologic Involvement And Neutropenia

3-Methylglutaconic Aciduria Type 7	Mga7
3-Methylglutaconic Aciduria Type Vii	Megcann
Mgca7	3-Methylglutaconic Aciduria-Cataract-Neurologic Involvement-Neutropenia Syndrome
3-Methylglutaconic Aciduria With Cataracts, Neurologic Involvement, And Neutropenia

Parkinsonism

Parkinsonism-Plus	Idiopathic Parkinsonism
Primary Parkinsonism	Paralysis Agitans Syndrome
Parkinsonian Syndrome	Trembling Paralysis
Paralysis Agitans	Shaking Palsy
Shaking Paralysis

Parkinson Disease, Late-Onset

Parkinson Disease	Parkinson'S Disease
PD	PARK
Parkinson Disease, Susceptibility To	Late Onset Parkinson'S Disease
Late Onset Parkinson Disease	Paralysis Agitans
Primary Parkinsonism	Idiopathic Parkinson Disease
Parkinson'S	Parkinson Disease, Late-Onset, Susceptibility To
Parkinson Disease, Age Of Onset, Modifier	Lewy Body Parkinson Disease
Idiopathic Parkinson'S Disease	Pd - [Parkinson Disease]
Parkinson Disease Nos	Parkinson, Nos
Primary Parkinson Disease

Combat Disorder

Combat Disorders

Combat Neurosis

3-Methylglutaconic Aciduria, Type Iii

Optic Atrophy	3-Methylglutaconic Aciduria Type 3
Costeff Syndrome	Mga3
Costeff Optic Atrophy Syndrome	Optic Atrophy Plus Syndrome
Infantile Optic Atrophy With Chorea And Spastic Paraplegia	3-Methylglutaconic Aciduria Type Iii
Autosomal Recessive Optic Atrophy Plus Syndrome	Autosomal Recessive Optic Atrophy Type 3
Opa3 Defect	MGCA3
Mga, Type Iii	Iraqi Jewish Optic Atrophy Plus
Mga Type Iii	Optic Atrophy, Infantile, With Chorea And Spastic Paraplegia
Iraqi-Jewish 'Optic Atrophy Plus'	Optic Atrophy 3, Autosomal Recessive
Opa3, Autosomal Recessive	Opa3-Related 3-Methylglutaconic Aciduria
Iraqi-Jewish Optic Atrophy Plus	Atrophy Of Optic Disc
3-Alpha Methylglutaconic Aciduria Type Iii	Optic Atrophy 3
Optic Atrophy Infantile With Chorea And Spastic Paraplegia	Autosomal Recessive Opa3
Autosomal Recessive Optic Atrophy 3	3-Methylglutaconic Aciduria 3
3-Alpha-Methylglutaconic Aciduria Type 3	Optic Atrophy 3 Autosomal Recessive
Atrophy, Optic	Atrophy, Optic, Plus Syndrome
Optic Nerve Atrophy	Primary Optic Atrophy
Oa - [Optic Atrophy]	Second Cranial Nerve Atrophy
Second Cranium Nerve Atrophy

Movement Disease

Movement Disorders

Movement Disorder

Microcephaly

Microencephaly	Microcephalus
Microcephalic	Nanocephaly
Congenital Microcephaly	Brain Hypoplasia
Brain Nondevelopment	Cephalic Hypoplasia
Undeveloped Cerebrum	Undeveloped Brain
Micrencephalon	Micrencephaly

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 (2)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-125959	Ucf-101	Inhibitor	98.06%	Unkown
HY-RS06487	HTRA2 Human Pre-designed siRNA Set A	Pre-designed Sets	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Canis familiaris	HTRA2	VGNC	VGNC:41836
Rattus norvegicus	HTRA2	RGD	RGD:1308906
Felis catus	HTRA2	VGNC	VGNC:62855
Macaca mulatta	HTRA2	VGNC	VGNC:73390
Bos taurus	HTRA2	VGNC	VGNC:30005
Mus musculus	HTRA2	MGD	MGI:1928676
Others	HTRA2	NCBI

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