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    Chchd10 coiled-coil-helix-coiled-coil-helix domain containing 10 [ Mus musculus (house mouse) ]

    Gene ID: 103172, updated on 5-Mar-2024

    Summary

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    Official Symbol
    Chchd10provided by MGI
    Official Full Name
    coiled-coil-helix-coiled-coil-helix domain containing 10provided by MGI
    Primary source
    MGI:MGI:2143558
    See related
    Ensembl:ENSMUSG00000049422 AllianceGenome:MGI:2143558
    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
    Ndg2; 1620401E04Rik
    Summary
    Involved in several processes, including mitochondria-nucleus signaling pathway; positive regulation of mitochondrial transcription; and stabilization of membrane potential. Located in mitochondrion. Is expressed in several structures, including brain ventricle and choroid plexus; early embryo; hypothalamus mantle layer; renal calyx; and ventral grey horn. Human ortholog(s) of this gene implicated in frontotemporal dementia and/or amyotrophic lateral sclerosis-2. Orthologous to human CHCHD10 (coiled-coil-helix-coiled-coil-helix domain containing 10). [provided by Alliance of Genome Resources, Apr 2022]
    Expression
    Biased expression in adrenal adult (RPKM 1343.9), duodenum adult (RPKM 646.7) and 10 other tissues See more
    Orthologs
    human all
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    Genomic context

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    See Chchd10 in Genome Data Viewer
    Location:
    10 C1; 10 38.62 cM
    Exon count:
    6
    Annotation release Status Assembly Chr Location
    RS_2024_02 current GRCm39 (GCF_000001635.27) 10 NC_000076.7 (75768964..75773584)
    108.20200622 previous assembly GRCm38.p6 (GCF_000001635.26) 10 NC_000076.6 (75933130..75937750)

    Chromosome 10 - NC_000076.7Genomic Context describing neighboring genes Neighboring gene SWI/SNF related, matrix associated, actin dependent regulator of chromatin, subfamily b, member 1 Neighboring gene matrix metallopeptidase 11 Neighboring gene STARR-positive B cell enhancer ABC_E8863 Neighboring gene STARR-seq mESC enhancer starr_27166 Neighboring gene predicted gene 867 Neighboring gene STARR-positive B cell enhancer ABC_E11443 Neighboring gene STARR-positive B cell enhancer ABC_E5150 Neighboring gene V-set pre-B cell surrogate light chain 3 Neighboring gene 60S ribosomal protein L27 pseudogene Neighboring gene predicted gene, 40698 Neighboring gene predicted gene 5134

    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. Mutant CHCHD10 causes an extensive metabolic rewiring that precedes OXPHOS dysfunction in a murine model of mitochondrial cardiomyopathy. Sayles NM, et al. Cell Rep, 2022 Mar 8. PMID 35263592, Free PMC Article
    2. Early death of ALS-linked CHCHD10-R15L transgenic mice with central nervous system, skeletal muscle, and cardiac pathology. Ryan ÉB, et al. iScience, 2021 Feb 19. PMID 33659869, Free PMC Article
    3. ALS/FTD mutant CHCHD10 mice reveal a tissue-specific toxic gain-of-function and mitochondrial stress response. Anderson CJ, et al. Acta Neuropathol, 2019 Jul. PMID 30877432, Free PMC Article
    4. Mitochondrial defect in muscle precedes neuromuscular junction degeneration and motor neuron death in CHCHD10(S59L/+) mouse. Genin EC, et al. Acta Neuropathol, 2019 Jul. PMID 30874923
    5. Nur77 gene knockout alters dopamine neuron biochemical activity and dopamine turnover. Gilbert F, et al. Biol Psychiatry, 2006 Sep 15. PMID 16893530, Free PMC Article

    See all (31) citations in PubMed

    GeneRIFs: Gene References Into Functions

    What's a GeneRIF?
    1. Mitochondrial protein CHCHD10 inhibits NDV replication and reduces pathological changes.
      Title: Mitochondrial protein CHCHD10 inhibits NDV replication and reduces pathological changes.
    2. CHCHD10 mutations induce tissue-specific mitochondrial DNA deletions with a distinct signature.
      Title: CHCHD10 mutations induce tissue-specific mitochondrial DNA deletions with a distinct signature.
    3. Mutant CHCHD10 causes an extensive metabolic rewiring that precedes OXPHOS dysfunction in a murine model of mitochondrial cardiomyopathy.
      Title: Mutant CHCHD10 causes an extensive metabolic rewiring that precedes OXPHOS dysfunction in a murine model of mitochondrial cardiomyopathy.
    4. Loss of mitochondrial protein CHCHD10 in skeletal muscle causes neuromuscular junction impairment.
      Title: Loss of mitochondrial protein CHCHD10 in skeletal muscle causes neuromuscular junction impairment.
    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. CHCHD10 is highly expressed in the neuromuscular junction post-synaptic part
      Title: Mitochondrial defect in muscle precedes neuromuscular junction degeneration and motor neuron death in CHCHD10(S59L/+) mouse.
    7. In the presence of HA1004, NBRE binding by Nur77 protein increases with time (6 h vs 2 h), whereas the final outcome of both inhibitors is apoptosis of thymic lymphoma cells.
      Title: Nur77 nuclear import and its NBRE-binding activity in thymic lymphoma cells are regulated by different mechanisms sensitive to FK506 or HA1004.
    8. Nur77 gene knockout alters dopamine neuron biochemical activity and dopamine turnover.
      Title: Nur77 gene knockout alters dopamine neuron biochemical activity and dopamine turnover.
    Submit: New GeneRIF Correction

    Variation

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    Alleles

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

    General gene information

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    Markers

    Homology

    Gene Ontology Provided by MGI

    Function Evidence Code Pubs
    enables protein binding IPI
    Inferred from Physical Interaction
    more info
    		 PubMed 
    Process Evidence Code Pubs
    involved_in maintenance of protein location in nucleus IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    involved_in maintenance of synapse structure IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    involved_in mitochondria-nucleus signaling pathway IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    involved_in mitochondrial nucleoid organization ISO
    Inferred from Sequence Orthology
    more info
    		  
    involved_in mitochondrion organization IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    involved_in mitochondrion organization IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    involved_in mitochondrion organization ISO
    Inferred from Sequence Orthology
    more info
    		  
    involved_in oxidative phosphorylation ISO
    Inferred from Sequence Orthology
    more info
    		  
    involved_in positive regulation of cristae formation ISO
    Inferred from Sequence Orthology
    more info
    		  
    involved_in positive regulation of mitochondrial outer membrane permeabilization involved in apoptotic signaling pathway ISO
    Inferred from Sequence Orthology
    more info
    		  
    involved_in positive regulation of mitochondrial transcription IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    involved_in protein-containing complex assembly ISO
    Inferred from Sequence Orthology
    more info
    		  
    involved_in stabilization of membrane potential IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    Component Evidence Code Pubs
    part_of MICOS complex ISO
    Inferred from Sequence Orthology
    more info
    		  
    located_in mitochondrial intermembrane space ISO
    Inferred from Sequence Orthology
    more info
    		  
    located_in mitochondrion HDA PubMed 
    is_active_in mitochondrion IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    located_in mitochondrion IDA
    Inferred from Direct Assay
    more info
    		 PubMed 
    located_in mitochondrion IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    located_in mitochondrion ISO
    Inferred from Sequence Orthology
    more info
    		  
    is_active_in nucleus IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  

    General protein information

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    Preferred Names
    coiled-coil-helix-coiled-coil-helix domain-containing protein 10, mitochondrial
    Names
    Nur77 downstream gene 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_175329.4 → NP_780538.2  coiled-coil-helix-coiled-coil-helix domain-containing protein 10, mitochondrial

      See identical proteins and their annotated locations for NP_780538.2

      Status: VALIDATED

      Source sequence(s)
      BC083357, BM934773, W16247
      Consensus CDS
      CCDS23938.1
      UniProtKB/TrEMBL
      Q7TNL9, Q8BW15
      Related
      ENSMUSP00000151675.2, ENSMUST00000219839.2

    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_000076.7 Reference GRCm39 C57BL/6J

      Range
      75768964..75773584
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. XM_036155455.1 → XP_036011348.1  coiled-coil-helix-coiled-coil-helix domain-containing protein 10, mitochondrial isoform X1

      UniProtKB/TrEMBL
      Q7TNL9, Q8BW15
      Related
      ENSMUSP00000059575.7, ENSMUST00000058906.7
    Nucleotide Protein Strain
    Heading Accession and Version

    Gene LinkOut

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