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

    Gene ID: 67013, updated on 27-Nov-2024

    Summary

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    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, Nov 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

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    See Oma1 in Genome Data Viewer
    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_000070.7Genomic Context describing neighboring genes Neighboring gene RIKEN cDNA 4921539E11 gene Neighboring gene STARR-seq mESC enhancer starr_10938 Neighboring gene STARR-seq mESC enhancer starr_10939 Neighboring gene RIKEN cDNA 4930456L15 gene Neighboring gene CapStarr-seq enhancer MGSCv37_chr4:102986166-102986353 Neighboring gene predicted gene, 36523 Neighboring gene predicted gene 12718 Neighboring gene STARR-seq mESC enhancer starr_10940 Neighboring gene STARR-seq mESC enhancer starr_10941 Neighboring gene NADH dehydrogenase (ubiquinone) Fe-S protein 2 pseudogene

    Genomic regions, transcripts, and products

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    Go to nucleotide: Graphics FASTA GenBank

    Expression

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    • 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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    Related articles in PubMed

    1. OMA1-mediated integrated stress response protects against ferroptosis in mitochondrial cardiomyopathy. Ahola S, et al. Cell Metab, 2022 Nov 1. PMID 36113464
    2. Ablation of the stress protease OMA1 protects against heart failure in mice. Acin-Perez R, et al. Sci Transl Med, 2018 Mar 28. PMID 29593106
    3. EGCG protects cardiomyocytes against hypoxia-reperfusion injury through inhibition of OMA1 activation. Nan J, et al. J Cell Sci, 2019 Jan 25. PMID 30518622
    4. Stress-induced OMA1 activation and autocatalytic turnover regulate OPA1-dependent mitochondrial dynamics. Baker MJ, et al. EMBO J, 2014 Mar 18. PMID 24550258, Free PMC Article
    5. OMA1 mediates OPA1 proteolysis and mitochondrial fragmentation in experimental models of ischemic kidney injury. Xiao X, et al. Am J Physiol Renal Physiol, 2014 Jun 1. PMID 24671334, Free PMC Article

    See all (41) citations in PubMed

    GeneRIFs: Gene References Into Functions

    What's a GeneRIF?
    1. 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.
    2. An OMA1 redox site controls mitochondrial homeostasis, sarcoma growth, and immunogenicity.
      Title: An OMA1 redox site controls mitochondrial homeostasis, sarcoma growth, and immunogenicity.
    3. OMA1-mediated integrated stress response protects against ferroptosis in mitochondrial cardiomyopathy.
      Title: OMA1-mediated integrated stress response protects against ferroptosis in mitochondrial cardiomyopathy.
    4. 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.
    5. 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.
    6. 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.
    7. 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.
    8. 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.
    9. 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.
    10. 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

    Pathways from PubChem

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    Interactions

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    Products Interactant Other Gene Complex Source Pubs Description

    General gene information

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    Homology

    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 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
    NP_001406044.1
    NP_001406045.1
    NP_001406046.1
    NP_001406047.1
    NP_001406048.1
    NP_001406049.1
    NP_001406050.1
    NP_001406051.1
    NP_001406052.1
    NP_001406053.1
    NP_001406054.1
    NP_001406055.1
    NP_001406056.1
    NP_001406057.1
    NP_001406058.1
    NP_001406059.1
    NP_001406060.1
    NP_001406061.1
    NP_080185.1
    XP_006503362.1
    XP_017175849.1
    XP_030109574.1
    XP_036020249.1
    XP_036020251.1

    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)

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

      Status: VALIDATED

      Source sequence(s)
      AL772338
      UniProtKB/Swiss-Prot
      Q3UWN9, Q9D8H7

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

      Status: VALIDATED

      Source sequence(s)
      AL772338
      UniProtKB/Swiss-Prot
      Q3UWN9, Q9D8H7

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

      Status: VALIDATED

      Source sequence(s)
      AL772338

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

      Status: VALIDATED

      Source sequence(s)
      AL772338

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

      Status: VALIDATED

      Source sequence(s)
      AL772338

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

      Status: VALIDATED

      Source sequence(s)
      AL772338

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

      Status: VALIDATED

      Source sequence(s)
      AL772338

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

      Status: VALIDATED

      Source sequence(s)
      AL772338
      UniProtKB/Swiss-Prot
      Q3UWN9, Q9D8H7

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

      Status: VALIDATED

      Source sequence(s)
      AL772338

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

      Status: VALIDATED

      Source sequence(s)
      AL772338

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

      Status: VALIDATED

      Source sequence(s)
      AL772338

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

      Status: VALIDATED

      Source sequence(s)
      AL772338

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

      Status: VALIDATED

      Source sequence(s)
      AL772338

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

      Status: VALIDATED

      Source sequence(s)
      AL772338

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

      Status: VALIDATED

      Source sequence(s)
      AL772338

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

      Status: VALIDATED

      Source sequence(s)
      AL772338

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

      Status: VALIDATED

      Source sequence(s)
      AL772338

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

      Status: VALIDATED

      Source sequence(s)
      AL772338

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

      Status: VALIDATED

      Source sequence(s)
      AL772338

    20. 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

    1. NC_000070.7 Reference GRCm39 C57BL/6J

      Range
      103170991..103229066
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. 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

    2. 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

    3. 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

    4. 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

    5. 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

    RNA

    1. XR_867888.1 RNA Sequence

    Nucleotide Protein Strain
    Heading Accession and Version
    Protein Accession Links
    GenPept Link UniProtKB Link
    Q9D8H7.1 GenPept UniProtKB/Swiss-Prot:Q9D8H7

    Gene LinkOut

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