Oma1 OMA1 zinc metallopeptidase [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: Oma1provided by MGI
Official Full Name: OMA1 zinc metallopeptidaseprovided by MGI
Primary source: MGI:MGI:1914263
See related: Ensembl:ENSMUSG00000035069 AllianceGenome:MGI:1914263
Gene type: protein coding
RefSeq status: VALIDATED
Organism: Mus musculus
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as: MPRP-1; ZMPOMA1; 2010001O09Rik
Summary: Enables metalloendopeptidase activity. Involved in several processes, including diet induced thermogenesis; negative regulation of mitochondrial fusion; and protein processing. Acts upstream of or within cristae formation. Located in mitochondrial inner membrane. Is expressed in alimentary system; cerebral cortex; liver lobe; nose; and submandibular gland primordium. Orthologous to human OMA1 (OMA1 zinc metallopeptidase). [provided by Alliance of Genome Resources, Dec 2024]
Expression: Ubiquitous expression in bladder adult (RPKM 2.1), heart adult (RPKM 1.7) and 28 other tissues See more
Orthologs: human all
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Genomic context

Location:: 4 C6; 4 47.6 cM

Exon count:: 11

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	4	NC_000070.7 (103170991..103229066)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	4	NC_000070.6 (103311875..103371869)

Chromosome 4 - NC_000070.7 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Go to reference sequence details

Genomic Sequence:

Go to nucleotide: Graphics FASTA GenBank

Expression

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See details

Project title: Mouse ENCODE transcriptome data
Description: RNA profiling data sets generated by the Mouse ENCODE project.
BioProject: PRJNA66167
Publication: PMID 25409824
Analysis date: n/a

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

Loss of UCP2 causes mitochondrial fragmentation by OMA1-dependent proteolytic processing of OPA1 in podocytes.
Title: Loss of UCP2 causes mitochondrial fragmentation by OMA1-dependent proteolytic processing of OPA1 in podocytes.
An OMA1 redox site controls mitochondrial homeostasis, sarcoma growth, and immunogenicity.
Title: An OMA1 redox site controls mitochondrial homeostasis, sarcoma growth, and immunogenicity.
OMA1-mediated integrated stress response protects against ferroptosis in mitochondrial cardiomyopathy.
Title: OMA1-mediated integrated stress response protects against ferroptosis in mitochondrial cardiomyopathy.
OMA1 mediates local and global stress responses against protein misfolding in CHCHD10 mitochondrial myopathy.
Title: OMA1 mediates local and global stress responses against protein misfolding in CHCHD10 mitochondrial myopathy.
Loss of CHCHD2 and CHCHD10 activates OMA1 peptidase to disrupt mitochondrial cristae phenocopying patient mutations.
Title: Loss of CHCHD2 and CHCHD10 activates OMA1 peptidase to disrupt mitochondrial cristae phenocopying patient mutations.
Mitochondrial Safeguard: a stress response that offsets extreme fusion and protects respiratory function via flickering-induced Oma1 activation.
Title: Mitochondrial Safeguard: a stress response that offsets extreme fusion and protects respiratory function via flickering-induced Oma1 activation.
EGCG directly interacted with OMA1 and potently inhibited self-cleavage of OMA1, leading to attenuated OPA1 cleavage. This study, therefore, suggests to use OMA1 inhibition induced by EGCG to treat cardiac hypoxia-reperfusion injury.
Title: EGCG protects cardiomyocytes against hypoxia-reperfusion injury through inhibition of OMA1 activation.
The m-AAA protease associated with neurodegeneration limits MCU activity in mitochondria.
Title: The m-AAA Protease Associated with Neurodegeneration Limits MCU Activity in Mitochondria.
stress-induced OMA1 activation and guanosine triphosphatase OPA1 cleavage limit mitochondrial fusion and promote neuronal death
Title: Loss of OMA1 delays neurodegeneration by preventing stress-induced OPA1 processing in mitochondria.
The IMQC protease Oma1 is required for the stability of the respiratory supercomplexes and thus balanced and tunable bioenergetic function.
Title: Metalloprotease OMA1 Fine-tunes Mitochondrial Bioenergetic Function and Respiratory Supercomplex Stability.

Submit: New GeneRIF Correction See all GeneRIFs (15)

Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics (MGI)

Endonuclease-mediated (1)
Targeted (3) 1 citation

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables lipid binding	IEA Inferred from Electronic Annotation more info
enables metal ion binding	IEA Inferred from Electronic Annotation more info
enables metalloendopeptidase activity	IBA Inferred from Biological aspect of Ancestor more info
enables metalloendopeptidase activity	IDA Inferred from Direct Assay more info	PubMed
enables metalloendopeptidase activity	IMP Inferred from Mutant Phenotype more info	PubMed
enables metalloendopeptidase activity	ISO Inferred from Sequence Orthology more info

Process	Evidence Code	Pubs
involved_in HRI-mediated signaling	ISO Inferred from Sequence Orthology more info
involved_in HRI-mediated signaling	ISS Inferred from Sequence or Structural Similarity more info
acts_upstream_of_or_within cristae formation	IGI Inferred from Genetic Interaction more info	PubMed
involved_in diet induced thermogenesis	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in energy homeostasis	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in glucose metabolic process	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in integrated stress response signaling	ISO Inferred from Sequence Orthology more info
involved_in integrated stress response signaling	ISS Inferred from Sequence or Structural Similarity more info
involved_in lipid metabolic process	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in mitochondrial protein processing	IBA Inferred from Biological aspect of Ancestor more info
involved_in mitochondrial protein processing	IDA Inferred from Direct Assay more info	PubMed
acts_upstream_of_or_within mitochondrial protein processing	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in mitochondrial protein processing	ISO Inferred from Sequence Orthology more info
involved_in mitochondrial protein processing	ISS Inferred from Sequence or Structural Similarity more info
involved_in mitochondrial respiratory chain complex assembly	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within mitochondrion organization	IGI Inferred from Genetic Interaction more info	PubMed
involved_in negative regulation of mitochondrial fusion	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in negative regulation of mitochondrial fusion	ISO Inferred from Sequence Orthology more info
involved_in positive regulation of apoptotic process	ISO Inferred from Sequence Orthology more info
involved_in positive regulation of apoptotic process	ISS Inferred from Sequence or Structural Similarity more info
involved_in positive regulation of cold-induced thermogenesis	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in protein autoprocessing	IDA Inferred from Direct Assay more info	PubMed
involved_in protein quality control for misfolded or incompletely synthesized proteins	IBA Inferred from Biological aspect of Ancestor more info
involved_in protein quality control for misfolded or incompletely synthesized proteins	IEA Inferred from Electronic Annotation more info
involved_in protein quality control for misfolded or incompletely synthesized proteins	ISO Inferred from Sequence Orthology more info
involved_in regulation of cristae formation	ISO Inferred from Sequence Orthology more info
involved_in regulation of cristae formation	ISS Inferred from Sequence or Structural Similarity more info
involved_in zymogen activation	IDA Inferred from Direct Assay more info	PubMed

Component	Evidence Code	Pubs
is_active_in mitochondrial inner membrane	IBA Inferred from Biological aspect of Ancestor more info
located_in mitochondrial inner membrane	IDA Inferred from Direct Assay more info	PubMed
located_in mitochondrial inner membrane	ISO Inferred from Sequence Orthology more info
located_in mitochondrial inner membrane	TAS Traceable Author Statement more info
located_in mitochondrial intermembrane space	IEA Inferred from Electronic Annotation more info
is_active_in mitochondrial intermembrane space	ISO Inferred from Sequence Orthology more info
located_in mitochondrial membrane	ISO Inferred from Sequence Orthology more info
located_in mitochondrion	HDA more info	PubMed
located_in mitochondrion	IDA Inferred from Direct Assay more info	PubMed

General protein information

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Preferred Names: metalloendopeptidase OMA1, mitochondrial

Names: OMA1 homolog, zinc metallopeptidase; overlapping with the m-AAA protease 1 homolog

NP_001406043.1

EC 3.4.24.-

NP_001406044.1

EC 3.4.24.-

NP_001406045.1

EC 3.4.24.-

NP_001406046.1

EC 3.4.24.-

NP_001406047.1

EC 3.4.24.-

NP_001406048.1

EC 3.4.24.-

NP_001406049.1

EC 3.4.24.-

NP_001406050.1

EC 3.4.24.-

NP_001406051.1

EC 3.4.24.-

NP_001406052.1

EC 3.4.24.-

NP_001406053.1

EC 3.4.24.-

NP_001406054.1

EC 3.4.24.-

NP_001406055.1

EC 3.4.24.-

NP_001406056.1

EC 3.4.24.-

NP_001406057.1

EC 3.4.24.-

NP_001406058.1

EC 3.4.24.-

NP_001406059.1

EC 3.4.24.-

NP_001406060.1

EC 3.4.24.-

NP_001406061.1

EC 3.4.24.-

NP_080185.1

EC 3.4.24.-

XP_006503362.1

EC 3.4.24.-

XP_017175849.1

EC 3.4.24.-

XP_030109574.1

EC 3.4.24.-

XP_036020249.1

EC 3.4.24.-

XP_036020251.1

EC 3.4.24.-

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

NM_001419114.1 → NP_001406043.1 metalloendopeptidase OMA1, mitochondrial isoform a precursor

Status: VALIDATED

Source sequence(s)

AL772338

UniProtKB/Swiss-Prot

Q3UWN9, Q9D8H7
NM_001419115.1 → NP_001406044.1 metalloendopeptidase OMA1, mitochondrial isoform a precursor

Status: VALIDATED

Source sequence(s)

AL772338

UniProtKB/Swiss-Prot

Q3UWN9, Q9D8H7
NM_001419116.1 → NP_001406045.1 metalloendopeptidase OMA1, mitochondrial isoform b precursor

Status: VALIDATED

Source sequence(s)

AL772338
NM_001419117.1 → NP_001406046.1 metalloendopeptidase OMA1, mitochondrial isoform b precursor

Status: VALIDATED

Source sequence(s)

AL772338
NM_001419118.1 → NP_001406047.1 metalloendopeptidase OMA1, mitochondrial isoform b precursor

Status: VALIDATED

Source sequence(s)

AL772338
NM_001419119.1 → NP_001406048.1 metalloendopeptidase OMA1, mitochondrial isoform c precursor

Status: VALIDATED

Source sequence(s)

AL772338
NM_001419120.1 → NP_001406049.1 metalloendopeptidase OMA1, mitochondrial isoform d precursor

Status: VALIDATED

Source sequence(s)

AL772338
NM_001419121.1 → NP_001406050.1 metalloendopeptidase OMA1, mitochondrial isoform e precursor

Status: VALIDATED

Source sequence(s)

AL772338

UniProtKB/Swiss-Prot

Q3UWN9, Q9D8H7
NM_001419122.1 → NP_001406051.1 metalloendopeptidase OMA1, mitochondrial isoform f precursor

Status: VALIDATED

Source sequence(s)

AL772338
NM_001419123.1 → NP_001406052.1 metalloendopeptidase OMA1, mitochondrial isoform g precursor

Status: VALIDATED

Source sequence(s)

AL772338
NM_001419124.1 → NP_001406053.1 metalloendopeptidase OMA1, mitochondrial isoform h precursor

Status: VALIDATED

Source sequence(s)

AL772338
NM_001419125.1 → NP_001406054.1 metalloendopeptidase OMA1, mitochondrial isoform i precursor

Status: VALIDATED

Source sequence(s)

AL772338
NM_001419126.1 → NP_001406055.1 metalloendopeptidase OMA1, mitochondrial isoform j

Status: VALIDATED

Source sequence(s)

AL772338
NM_001419127.1 → NP_001406056.1 metalloendopeptidase OMA1, mitochondrial isoform j

Status: VALIDATED

Source sequence(s)

AL772338
NM_001419128.1 → NP_001406057.1 metalloendopeptidase OMA1, mitochondrial isoform j

Status: VALIDATED

Source sequence(s)

AL772338
NM_001419129.1 → NP_001406058.1 metalloendopeptidase OMA1, mitochondrial isoform j

Status: VALIDATED

Source sequence(s)

AL772338
NM_001419130.1 → NP_001406059.1 metalloendopeptidase OMA1, mitochondrial isoform k

Status: VALIDATED

Source sequence(s)

AL772338
NM_001419131.1 → NP_001406060.1 metalloendopeptidase OMA1, mitochondrial isoform k

Status: VALIDATED

Source sequence(s)

AL772338
NM_001419132.1 → NP_001406061.1 metalloendopeptidase OMA1, mitochondrial isoform l

Status: VALIDATED

Source sequence(s)

AL772338
NM_025909.4 → NP_080185.1 metalloendopeptidase OMA1, mitochondrial isoform a precursor

See identical proteins and their annotated locations for NP_080185.1

Status: VALIDATED

Source sequence(s)

AL772338

Consensus CDS

CCDS18412.1

UniProtKB/Swiss-Prot

Q3UWN9, Q9D8H7

Related

ENSMUSP00000045269.4, ENSMUST00000035780.4

Conserved Domains (1) summary

cd07331
Location:265 → 452

M48C_Oma1_like; Peptidase M48C, integral membrane endopeptidase

RefSeqs of Annotated Genomes: GCF_000001635.27-RS_2024_02

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCm39 C57BL/6J

Genomic

NC_000070.7 Reference GRCm39 C57BL/6J

Range

103170991..103229066

Download

GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

XM_017320360.1 → XP_017175849.1 metalloendopeptidase OMA1, mitochondrial isoform X4

Conserved Domains (1) summary

cd07331
Location:265 → 359

M48C_Oma1_like; Peptidase M48C, integral membrane endopeptidase
XM_036164356.1 → XP_036020249.1 metalloendopeptidase OMA1, mitochondrial isoform X3

Conserved Domains (1) summary

cd07331
Location:265 → 402

M48C_Oma1_like; Peptidase M48C, integral membrane endopeptidase
XM_030253714.2 → XP_030109574.1 metalloendopeptidase OMA1, mitochondrial isoform X8

Conserved Domains (1) summary

cd07331
Location:25 → 169

M48C_Oma1_like; Peptidase M48C, integral membrane endopeptidase
XM_006503299.5 → XP_006503362.1 metalloendopeptidase OMA1, mitochondrial isoform X1

See identical proteins and their annotated locations for XP_006503362.1

UniProtKB/Swiss-Prot

Q3UWN9, Q9D8H7

Conserved Domains (1) summary

cd07331
Location:265 → 452

M48C_Oma1_like; Peptidase M48C, integral membrane endopeptidase
XM_036164358.1 → XP_036020251.1 metalloendopeptidase OMA1, mitochondrial isoform X7

Conserved Domains (1) summary

cd07331
Location:25 → 212

M48C_Oma1_like; Peptidase M48C, integral membrane endopeptidase