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Opa1 OPA1, mitochondrial dynamin like GTPase [ Mus musculus (house mouse) ]

Gene ID: 74143, updated on 18-Apr-2024

Summary

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Official Symbol
Opa1provided by MGI
Official Full Name
OPA1, mitochondrial dynamin like GTPaseprovided by MGI
Primary source
MGI:MGI:1921393
See related
Ensembl:ENSMUSG00000038084 AllianceGenome:MGI:1921393
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
lilr3; mKIAA0567; 1200011N24Rik
Summary
Predicted to enable several functions, including GTPase activity; anion binding activity; and microtubule binding activity. Involved in negative regulation of endoplasmic reticulum stress-induced intrinsic apoptotic signaling pathway and negative regulation of release of cytochrome c from mitochondria. Acts upstream of or within mitochondrial fusion and neural tube closure. Located in dendrite; mitochondrial inner membrane; and mitochondrial intermembrane space. Is expressed in several structures, including heart; liver; lung; skin; and visual system. Used to study optic atrophy. Human ortholog(s) of this gene implicated in Behr syndrome; dominant optic atrophy plus syndrome; mitochondrial DNA depletion syndrome 14; optic atrophy; and optic atrophy 1. Orthologous to human OPA1 (OPA1 mitochondrial dynamin like GTPase). [provided by Alliance of Genome Resources, Apr 2022]
Expression
Ubiquitous expression in cerebellum adult (RPKM 11.0), CNS E18 (RPKM 10.5) and 28 other tissues See more
Orthologs
human all
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Genomic context

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Location:
16 B2; 16 20.65 cM
Exon count:
32
Annotation release Status Assembly Chr Location
RS_2024_02 current GRCm39 (GCF_000001635.27) 16 NC_000082.7 (29398099..29481924)
108.20200622 previous assembly GRCm38.p6 (GCF_000001635.26) 16 NC_000082.6 (29579281..29663127)

Chromosome 16 - NC_000082.7Genomic Context describing neighboring genes Neighboring gene phospholipase A and acyltransferase 1 Neighboring gene ATPase type 13A5 Neighboring gene STARR-seq mESC enhancer starr_40522 Neighboring gene STARR-seq mESC enhancer starr_40523 Neighboring gene ATPase type 13A4 Neighboring gene zinc finger protein 788 pseudogene Neighboring gene CapStarr-seq enhancer MGSCv37_chr16:29579308-29579525 Neighboring gene STARR-seq mESC enhancer starr_40527 Neighboring gene STARR-seq mESC enhancer starr_40530 Neighboring gene STARR-seq mESC enhancer starr_40532 Neighboring gene STARR-seq mESC enhancer starr_40534 Neighboring gene STARR-seq mESC enhancer starr_40540 Neighboring gene predicted gene, 32740 Neighboring gene predicted gene, 46567 Neighboring gene predicted gene, 32679

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. OPA1 Regulates Lipid Metabolism and Cold-Induced Browning of White Adipose Tissue in Mice. Pereira RO, et al. Diabetes, 2022 Dec 1. PMID 36170659, Free PMC Article
  2. L-OPA1 deficiency aggravates necroptosis of alveolar epithelial cells through impairing mitochondrial function during acute lung injury in mice. Jiang HL, et al. J Cell Physiol, 2022 Jul. PMID 35478455
  3. Ischemia-induced cleavage of OPA1 at S1 site aggravates mitochondrial fragmentation and reperfusion injury in neurons. Li X, et al. Cell Death Dis, 2022 Apr 8. PMID 35395832, Free PMC Article
  4. Disrupted circadian oscillations in type 2 diabetes are linked to altered rhythmic mitochondrial metabolism in skeletal muscle. Gabriel BM, et al. Sci Adv, 2021 Oct 22. PMID 34669477, Free PMC Article
  5. Progressive Mitochondrial SOD1(G93A) Accumulation Causes Severe Structural, Metabolic and Functional Aberrations through OPA1 Down-Regulation in a Mouse Model of Amyotrophic Lateral Sclerosis. Méndez-López I, et al. Int J Mol Sci, 2021 Jul 30. PMID 34360957, Free PMC Article

See all (178) citations in PubMed

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
  1. The human OPA1[delTTAG] mutation induces adult onset and progressive auditory neuropathy in mice.
    Title: The human OPA1(delTTAG) mutation induces adult onset and progressive auditory neuropathy in mice.
  2. GDF15 is required for cold-induced thermogenesis and contributes to improved systemic metabolic health following loss of OPA1 in brown adipocytes.
    Title: GDF15 is required for cold-induced thermogenesis and contributes to improved systemic metabolic health following loss of OPA1 in brown adipocytes.
  3. [Long noncoding RNA H19 promotes vascular calcification by repressing the Bax inhibitor 1/optic atrophy 1 pathway].
    Title: [Long noncoding RNA H19 promotes vascular calcification by repressing the Bax inhibitor 1/optic atrophy 1 pathway].
  4. 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.
  5. The mitochondrial fusion protein OPA1 is dispensable in the liver and its absence induces mitohormesis to protect liver from drug-induced injury.
    Title: The mitochondrial fusion protein OPA1 is dispensable in the liver and its absence induces mitohormesis to protect liver from drug-induced injury.
  6. Attenuation of PM2.5-induced alveolar epithelial cells and lung injury through regulation of mitochondrial fission and fusion.
    Title: Attenuation of PM(2.5)-induced alveolar epithelial cells and lung injury through regulation of mitochondrial fission and fusion.
  7. SMYD1a protects the heart from ischemic injury by regulating OPA1-mediated cristae remodeling and supercomplex formation.
    Title: SMYD1a protects the heart from ischemic injury by regulating OPA1-mediated cristae remodeling and supercomplex formation.
  8. BNP protects against diabetic cardiomyopathy by promoting Opa1-mediated mitochondrial fusion via activating the PKG-STAT3 pathway.
    Title: BNP protects against diabetic cardiomyopathy by promoting Opa1-mediated mitochondrial fusion via activating the PKG-STAT3 pathway.
  9. OPA1 Regulates Lipid Metabolism and Cold-Induced Browning of White Adipose Tissue in Mice.
    Title: OPA1 Regulates Lipid Metabolism and Cold-Induced Browning of White Adipose Tissue in Mice.
  10. L-OPA1 deficiency aggravates necroptosis of alveolar epithelial cells through impairing mitochondrial function during acute lung injury in mice.
    Title: L-OPA1 deficiency aggravates necroptosis of alveolar epithelial cells through impairing mitochondrial function during acute lung injury in mice.
Submit: New GeneRIF Correction See all GeneRIFs (89)

Variation

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Alleles

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

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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Markers

Homology

Gene Ontology Provided by MGI

Function Evidence Code Pubs
enables GTP binding ISS
Inferred from Sequence or Structural Similarity
more info
 PubMed 
enables GTPase activity IBA
Inferred from Biological aspect of Ancestor
more info
  
enables GTPase activity ISO
Inferred from Sequence Orthology
more info
  
enables GTPase activity TAS
Traceable Author Statement
more info
 PubMed 
enables cardiolipin binding ISO
Inferred from Sequence Orthology
more info
  
enables hydrolase activity IEA
Inferred from Electronic Annotation
more info
  
enables kinase binding ISO
Inferred from Sequence Orthology
more info
  
enables lipid binding IEA
Inferred from Electronic Annotation
more info
  
enables microtubule binding IBA
Inferred from Biological aspect of Ancestor
more info
  
enables nucleotide binding IEA
Inferred from Electronic Annotation
more info
  
enables phosphatidic acid binding ISO
Inferred from Sequence Orthology
more info
  
enables protein binding IPI
Inferred from Physical Interaction
more info
 PubMed 
enables protein-containing complex binding ISO
Inferred from Sequence Orthology
more info
  
Process Evidence Code Pubs
involved_in GTP metabolic process ISO
Inferred from Sequence Orthology
more info
  
acts_upstream_of_or_within apoptotic process IEA
Inferred from Electronic Annotation
more info
  
involved_in calcium import into the mitochondrion ISO
Inferred from Sequence Orthology
more info
  
involved_in cellular senescence ISO
Inferred from Sequence Orthology
more info
  
involved_in inner mitochondrial membrane organization ISO
Inferred from Sequence Orthology
more info
  
involved_in intracellular distribution of mitochondria ISO
Inferred from Sequence Orthology
more info
  
involved_in membrane tubulation ISO
Inferred from Sequence Orthology
more info
  
involved_in mitochondrial fusion IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in mitochondrial fusion IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in mitochondrial fusion ISO
Inferred from Sequence Orthology
more info
  
involved_in mitochondrial genome maintenance ISO
Inferred from Sequence Orthology
more info
  
acts_upstream_of_or_within mitochondrion organization ISO
Inferred from Sequence Orthology
more info
  
involved_in mitochondrion organization ISO
Inferred from Sequence Orthology
more info
  
involved_in mitochondrion organization NAS
Non-traceable Author Statement
more info
 PubMed 
involved_in negative regulation of apoptotic process ISO
Inferred from Sequence Orthology
more info
  
involved_in negative regulation of endoplasmic reticulum stress-induced intrinsic apoptotic signaling pathway IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in negative regulation of endoplasmic reticulum stress-induced intrinsic apoptotic signaling pathway ISO
Inferred from Sequence Orthology
more info
  
involved_in negative regulation of intrinsic apoptotic signaling pathway IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in negative regulation of release of cytochrome c from mitochondria IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in negative regulation of release of cytochrome c from mitochondria ISO
Inferred from Sequence Orthology
more info
  
acts_upstream_of_or_within neural tube closure IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in positive regulation of cellular response to insulin stimulus ISO
Inferred from Sequence Orthology
more info
  
involved_in positive regulation of dendrite development ISO
Inferred from Sequence Orthology
more info
  
involved_in positive regulation of dendritic spine morphogenesis ISO
Inferred from Sequence Orthology
more info
  
involved_in positive regulation of insulin receptor signaling pathway ISO
Inferred from Sequence Orthology
more info
  
involved_in positive regulation of mitochondrial fusion ISO
Inferred from Sequence Orthology
more info
  
involved_in positive regulation of neuron maturation ISO
Inferred from Sequence Orthology
more info
  
involved_in protein complex oligomerization ISO
Inferred from Sequence Orthology
more info
  
acts_upstream_of_or_within response to stimulus IEA
Inferred from Electronic Annotation
more info
  
involved_in visual perception ISO
Inferred from Sequence Orthology
more info
  
Component Evidence Code Pubs
is_active_in cytoplasm IBA
Inferred from Biological aspect of Ancestor
more info
  
located_in cytosol ISO
Inferred from Sequence Orthology
more info
  
located_in dendrite IDA
Inferred from Direct Assay
more info
 PubMed 
located_in membrane IEA
Inferred from Electronic Annotation
more info
  
is_active_in microtubule IBA
Inferred from Biological aspect of Ancestor
more info
  
located_in mitochondrial crista ISO
Inferred from Sequence Orthology
more info
  
located_in mitochondrial inner membrane HDA PubMed 
located_in mitochondrial inner membrane IDA
Inferred from Direct Assay
more info
 PubMed 
located_in mitochondrial inner membrane IMP
Inferred from Mutant Phenotype
more info
 PubMed 
located_in mitochondrial inner membrane ISO
Inferred from Sequence Orthology
more info
  
is_active_in mitochondrial intermembrane space IBA
Inferred from Biological aspect of Ancestor
more info
  
located_in mitochondrial intermembrane space IDA
Inferred from Direct Assay
more info
 PubMed 
located_in mitochondrial intermembrane space ISO
Inferred from Sequence Orthology
more info
  
is_active_in mitochondrial membrane IBA
Inferred from Biological aspect of Ancestor
more info
  
located_in mitochondrial outer membrane ISO
Inferred from Sequence Orthology
more info
  
located_in mitochondrion HDA PubMed 
located_in mitochondrion IDA
Inferred from Direct Assay
more info
 PubMed 
located_in mitochondrion ISO
Inferred from Sequence Orthology
more info
  
located_in nucleoplasm ISO
Inferred from Sequence Orthology
more info
  

General protein information

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Preferred Names
dynamin-like 120 kDa protein, mitochondrial
Names
large GTP-binding protein
largeG
optic atrophy 1 homolog
optic atrophy protein 1 homolog
NP_001186106.1
NP_001390099.1
NP_001390100.1
NP_001390101.1
NP_001390111.1
NP_598513.1
XP_006522718.2
XP_006522719.2

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_001199177.2 → NP_001186106.1  dynamin-like 120 kDa protein, mitochondrial isoform 1

    Status: VALIDATED

    Description
    Transcript Variant: This variant (1) represents the longer transcript and encodes the longer isoform (1).
    Source sequence(s)
    AC154438
    Consensus CDS
    CCDS57024.1
    UniProtKB/TrEMBL
    H7BX01, Q80TX7
    Related
    ENSMUSP00000036993.7, ENSMUST00000038867.13
    Conserved Domains (1) summary
    cd08771
    Location:305 → 579
    DLP_1; Dynamin_like protein family includes dynamins and Mx proteins

  2. NM_001403170.1 → NP_001390099.1  dynamin-like 120 kDa protein, mitochondrial isoform 3

    Status: VALIDATED

    Source sequence(s)
    AC154438

  3. NM_001403171.1 → NP_001390100.1  dynamin-like 120 kDa protein, mitochondrial isoform 4

    Status: VALIDATED

    Source sequence(s)
    AC154438
    Related
    ENSMUSP00000123880.2, ENSMUST00000161186.8

  4. NM_001403172.1 → NP_001390101.1  dynamin-like 120 kDa protein, mitochondrial isoform 4

    Status: VALIDATED

    Source sequence(s)
    AC154438

  5. NM_001403182.1 → NP_001390111.1  dynamin-like 120 kDa protein, mitochondrial isoform 2

    Status: VALIDATED

    Source sequence(s)
    AC154438
    UniProtKB/Swiss-Prot
    A6H6Q3, P58281, Q3ULA5, Q8BKU7, Q8BLL3, Q8BM08, Q8R3J7

  6. NM_133752.4 → NP_598513.1  dynamin-like 120 kDa protein, mitochondrial isoform 2

    See identical proteins and their annotated locations for NP_598513.1

    Status: VALIDATED

    Description
    Transcript Variant: This variant (2) lacks an in-frame exon in the 5' coding region, compared to variant 1. The resulting protein (isoform 2) is shorter, compared to isoform 1.
    Source sequence(s)
    AC154438
    Consensus CDS
    CCDS28096.1
    UniProtKB/Swiss-Prot
    A6H6Q3, P58281, Q3ULA5, Q8BKU7, Q8BLL3, Q8BM08, Q8R3J7
    UniProtKB/TrEMBL
    Q80TX7
    Related
    ENSMUSP00000124223.2, ENSMUST00000160597.8
    Conserved Domains (1) summary
    cd08771
    Location:287 → 561
    DLP_1; Dynamin_like protein family includes dynamins and Mx proteins

RNA

  1. NR_175360.1 RNA Sequence

    Status: VALIDATED

    Source sequence(s)
    AC154438

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_000082.7 Reference GRCm39 C57BL/6J

    Range
    29398099..29481924
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_006522656.5 → XP_006522719.2  dynamin-like 120 kDa protein, mitochondrial isoform X1

    UniProtKB/TrEMBL
    Q80TX7
    Conserved Domains (1) summary
    cd08771
    Location:427 → 701
    DLP_1; Dynamin_like protein family includes dynamins and Mx proteins

  2. XM_006522655.3 → XP_006522718.2  dynamin-like 120 kDa protein, mitochondrial isoform X1

    UniProtKB/TrEMBL
    Q80TX7
    Conserved Domains (1) summary
    cd08771
    Location:427 → 701
    DLP_1; Dynamin_like protein family includes dynamins and Mx proteins
Nucleotide Protein Strain
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
P58281.1 GenPept UniProtKB/Swiss-Prot:P58281

Gene LinkOut

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