OMA1 - OMA1 zinc metallopeptidase Gene

Also Known as DAB1; MPRP1; MPRP-1; YKR087C; ZMPOMA1; peptidase; 2010001O09Rik

Species: Homo sapiens

Gene Type: protein coding
Gene ID: 115209

About OMA1

Cytogenetic location: 1p32.2-p32.1 Genomic coordinates (GRCh38): 1:58,480,719-58,546,726 (from NCBI)

This gene has 11 transcripts (splice variants) and 198 orthologues. Ubiquitous expression in kidney (RPKM 9.7), prostate (RPKM 9.5) and 25 other tissues.

Summary

Enables metalloendopeptidase activity. Involved in several processes, including HRI-mediated signaling; proteolysis; and regulation of mitochondrion organization. Located in mitochondrial inner membrane. [provided by Alliance of Genome Resources, Apr 2022]

OMA1 Products (1)

mRNA Protein Name
NM_145243.5 NP_660286.1 metalloendopeptidase OMA1, mitochondrial precursor
Molecular Function GO Annotation Evidence References Source
enables metalloendopeptidase activity IDA
IDA: Inferred from direct assay
25275009 GOA
enables metalloendopeptidase activity IMP
IMP: Inferred from mutant phenotype
20038677 GOA
Biological Process GO Annotation Evidence References Source
involved in HRI-mediated signaling IDA
IDA: Inferred from direct assay
32132706 GOA
involved in cellular response to stress IDA
IDA: Inferred from direct assay
32132706 GOA
involved in integrated stress response signaling IDA
IDA: Inferred from direct assay
32132706 GOA
involved in mitochondrial protein processing IDA
IDA: Inferred from direct assay
25275009 GOA
involved in mitochondrial protein processing IMP
IMP: Inferred from mutant phenotype
25605331 GOA
involved in negative regulation of mitochondrial fusion IMP
IMP: Inferred from mutant phenotype
20038677 GOA
involved in positive regulation of apoptotic process IDA
IDA: Inferred from direct assay
25275009 GOA
involved in protein quality control for misfolded or incompletely synthesized proteins IMP
IMP: Inferred from mutant phenotype
20038677 GOA
involved in regulation of cristae formation IDA
IDA: Inferred from direct assay
25275009 GOA
Cellular Component GO Annotation Evidence References Source
located in mitochondrial inner membrane IDA
IDA: Inferred from direct assay
32132707 GOA
is active in mitochondrial intermembrane space IDA
IDA: Inferred from direct assay
32132706 GOA
located in mitochondrial membrane IDA
IDA: Inferred from direct assay
20038677 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

OMA1 Protein Structure

Peptidase_M48

Peptidase_M48: Peptidase family M48 (284 - 447)

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

metalloendopeptidase OMA1, mitochondrial

  • OMA1 homolog, zinc metallopeptidase

Recombinant OMA1 Proteins

Cat. No. Product Name Accession Purity
HY-P72010 OMA1 Protein, Human (Cell-Free, His-SUMO) Q96E52-1 (H14-S524) ≥ 90%, as determined by reducing SDS-PAGE.

Related Diseases

Diseases Alias
Spinocerebellar Ataxia 28
  • Spinocerebellar Ataxia Type 28

  • SCA28

  • Ataxia, Spinocerebellar, Type 28

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

Behr Syndrome
  • BEHRS

  • Abortive Cerebellar Ataxia

  • Optic Atrophy, Infantile Hereditary, With Neurologic Abnormalities

  • Optic Atrophy, Infantile Hereditary, Behr Complicated Form Of

  • Optic Atrophy In Early Childhood, Associated With Ataxia, Spasticity, Mental Retardation, And Posterior Column Sensory Loss

  • Obsolete: Behr Syndrome

  • Optic Atrophy In Early Childhood, Associated With Ataxia, Spasticity, Intellectual Disability, And Posterior Column Sensory Loss

  • Infantile Hereditary Optic Atrophy With Neurologic Abnormalities

Optic Atrophy 7 With Or Without Auditory Neuropathy
  • Optic Atrophy 7

  • OPA7

  • Autosomal Recessive Optic Atrophy, Opa7 Type

  • Optic Atrophy-7

  • Atrophy, Optic, Type 7, With/Without Auditory Neuropathy

Combined Oxidative Phosphorylation Deficiency 33
  • COXPD33

Optic Atrophy 11
  • OPA11

  • Atrophy, Optic, Type 11

Optic Nerve Disease
  • Optic Neuropathy

  • Disorder Of The Second Nerve

  • Optic Nerve Disorder

  • Optic Nerve

  • Abnormality Of The Optic Nerve

  • Optic Nerve Disorders

  • Neuropathy, Optic

  • Disorder Of The Optic Nerve

Spastic Ataxia
  • Spax

  • Ataxia, Spastic

Hereditary Spastic Paraplegia
  • Familial Spastic Paraplegia

  • Hereditary Spastic Paraparesis

  • Strumpell-Lorrain Disease

  • Familial Spastic Paraparesis

  • Hsp

  • Spg

  • Strümpell-Lorrain Disease

  • Spastic Paraplegia, Hereditary

  • French Settlement Disease

  • Strumpell-Lorrain Syndrome

  • Fsp

  • Spastic Paraplegia, Familial

  • Spastic Paraplegia Hereditary

  • Spastic Paraplegia 3, Autosomal Dominant

  • Spastic Paraparesis

  • Hereditary Spastic Paralysis

  • Familial Spastic Paralysis

  • Hereditary Spastic Ataxia

Dilated Cardiomyopathy
  • Familial Dilated Cardiomyopathy

  • Primary Dilated Cardiomyopathy

  • Idiopathic Dilated Cardiomyopathy

  • Congestive Cardiomyopathy

  • Idiopathic Dilation Cardiomyopathy

  • Primary Familial Dilated Cardiomyopathy

  • Cardiomyopathy, Dilated

  • DCM

  • Cardiomyopathy, Familial Dilated

  • Dilated Cardiomyopathy, Familial

  • Hypokinetic Dilated Cardiomyopathy, Familial

  • Familial Idiopathic Cardiomyopathy

  • Fdc

  • Cardiomyopathy, Familial Idiopathic

  • Idiopathic Cardiomegaly

  • Dilated Congestive Cardiomyopathy

  • Chronic Dilated Cardiomyopathy

  • Ccm - [Congestive Cardiomyopathy]

  • Cocm - [Congestive Cardiomyopathy]

  • Dcm - [Dilated Cardiomyopathy]

  • Dilated-Hypokinetic Cardiomyopathy

  • Congestive Idiopathic Cardiomyopathy

  • Primary Idiopathic Dilated Cardiomyopathy

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
Rattus norvegicus OMA1 RGD RGD:1304821
Bos taurus OMA1 VGNC VGNC:32428
Macaca mulatta OMA1 VGNC VGNC:75485
Canis familiaris OMA1 VGNC VGNC:44121
Mus musculus OMA1 MGD MGI:1914263
Felis catus OMA1 VGNC VGNC:63967
Others OMA1 NCBI