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    Auh AU RNA binding protein/enoyl-coenzyme A hydratase [ Mus musculus (house mouse) ]

    Gene ID: 11992, updated on 9-Dec-2024

    Summary

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    Official Symbol
    Auhprovided by MGI
    Official Full Name
    AU RNA binding protein/enoyl-coenzyme A hydrataseprovided by MGI
    Primary source
    MGI:MGI:1338011
    See related
    Ensembl:ENSMUSG00000021460 AllianceGenome:MGI:1338011
    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
    Summary
    Enables RNA binding activity and enoyl-CoA hydratase activity. Predicted to be involved in fatty acid beta-oxidation. Located in mitochondrion. Human ortholog(s) of this gene implicated in 3-methylglutaconic aciduria type 1 and renal tubular transport disease. Orthologous to human AUH (AU RNA binding methylglutaconyl-CoA hydratase). [provided by Alliance of Genome Resources, Dec 2024]
    Expression
    Ubiquitous expression in cerebellum adult (RPKM 10.0), kidney adult (RPKM 9.4) and 28 other tissues See more
    Orthologs
    human all
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    Genomic context

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    See Auh in Genome Data Viewer
    Location:
    13 A5- B1; 13 27.68 cM
    Exon count:
    14
    Annotation release Status Assembly Chr Location
    RS_2024_02 current GRCm39 (GCF_000001635.27) 13 NC_000079.7 (52989155..53083742, complement)
    108.20200622 previous assembly GRCm38.p6 (GCF_000001635.26) 13 NC_000079.6 (52835107..52929704, complement)

    Chromosome 13 - NC_000079.7Genomic Context describing neighboring genes Neighboring gene CapStarr-seq enhancer MGSCv37_chr13:52678844-52678953 Neighboring gene STARR-seq mESC enhancer starr_34550 Neighboring gene STARR-positive B cell enhancer ABC_E7238 Neighboring gene spleen tyrosine kinase Neighboring gene CapStarr-seq enhancer MGSCv37_chr13:52797592-52797775 Neighboring gene CapStarr-seq enhancer MGSCv37_chr13:52816883-52817066 Neighboring gene expressed sequence BB123696 Neighboring gene STARR-seq mESC enhancer starr_34551 Neighboring gene predicted gene, 33315 Neighboring gene STARR-seq mESC enhancer starr_34553 Neighboring gene STARR-positive B cell enhancer ABC_E3081 Neighboring gene STARR-positive B cell enhancer mm9_chr13:53024873-53025174 Neighboring gene STARR-seq mESC enhancer starr_34554 Neighboring gene STARR-positive B cell enhancer ABC_E4070 Neighboring gene predicted gene, 40950 Neighboring gene nuclear factor, interleukin 3, regulated

    Genomic regions, transcripts, and products

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    Go to nucleotide: Graphics FASTA GenBank

    Bibliography

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    Related articles in PubMed

    1. Characterisation and mitochondrial localisation of AUH, an AU-specific RNA-binding enoyl-CoA hydratase. Brennan LE, et al. Gene, 1999 Mar 4. PMID 10072761
    2. Characterization of the Cardiac Overexpression of HSPB2 Reveals Mitochondrial and Myogenic Roles Supported by a Cardiac HspB2 Interactome. Grose JH, et al. PLoS One, 2015. PMID 26465331, Free PMC Article
    3. ADAM10 hyperactivation acts on piccolo to deplete synaptic vesicle stores in Huntington's disease. Cozzolino F, et al. Hum Mol Genet, 2021 Jun 17. PMID 33601422
    4. Chemical Crosslinking Mass Spectrometry Analysis of Protein Conformations and Supercomplexes in Heart Tissue. Chavez JD, et al. Cell Syst, 2018 Jan 24. PMID 29199018, Free PMC Article
    5. The interactome of intact mitochondria by cross-linking mass spectrometry provides evidence for coexisting respiratory supercomplexes. Liu F, et al. Mol Cell Proteomics, 2018 Feb. PMID 29222160, Free PMC Article

    See all (26) citations in PubMed

    GeneRIFs: Gene References Into Functions

    Variation

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    Alleles

    Alleles of this type are documented at Mouse Genome Informatics  (MGI)
    • Endonuclease-mediated (4) 

    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 RNA binding IDA
    Inferred from Direct Assay
    more info
    		 PubMed 
    enables enoyl-CoA hydratase activity IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    enables enoyl-CoA hydratase activity IDA
    Inferred from Direct Assay
    more info
    		 PubMed 
    enables enoyl-CoA hydratase activity ISO
    Inferred from Sequence Orthology
    more info
    		  
    enables enoyl-CoA hydratase activity ISS
    Inferred from Sequence or Structural Similarity
    more info
    		  
    enables itaconyl-CoA hydratase activity IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables mRNA 3'-UTR binding ISO
    Inferred from Sequence Orthology
    more info
    		  
    enables mRNA 3'-UTR binding ISS
    Inferred from Sequence or Structural Similarity
    more info
    		  
    enables methylglutaconyl-CoA hydratase activity IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables methylglutaconyl-CoA hydratase activity ISO
    Inferred from Sequence Orthology
    more info
    		  
    Process Evidence Code Pubs
    involved_in L-leucine catabolic process IEA
    Inferred from Electronic Annotation
    more info
    		  
    involved_in fatty acid beta-oxidation IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    Component Evidence Code Pubs
    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 ISS
    Inferred from Sequence or Structural Similarity
    more info
    		  

    General protein information

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    Preferred Names
    methylglutaconyl-CoA hydratase, mitochondrial
    Names
    3-MG-CoA hydratase
    AU-binding enoyl-CoA hydratase
    AU-specific RNA-binding enoyl-CoA hydratase
    itaconyl-CoA hydratase
    muAUH
    NP_001413039.1
    NP_001413040.1
    NP_001413041.1
    NP_001413042.1
    NP_001413043.1
    NP_001413044.1
    NP_001413045.1
    NP_001413046.1
    NP_001413047.1
    NP_001413048.1
    NP_001413049.1
    NP_001413050.1
    NP_001413051.1
    NP_057918.2
    XP_006516909.1
    XP_017170862.1

    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_001426110.1 → NP_001413039.1  methylglutaconyl-CoA hydratase, mitochondrial isoform 2

      Status: VALIDATED

      Source sequence(s)
      AC140443, AC160765
      UniProtKB/Swiss-Prot
      Q80YD7, Q8QZS0, Q9CY78, Q9D155, Q9JLZ3

    2. NM_001426111.1 → NP_001413040.1  methylglutaconyl-CoA hydratase, mitochondrial isoform 3

      Status: VALIDATED

      Source sequence(s)
      AC140443, AC160765
      UniProtKB/Swiss-Prot
      Q80YD7, Q8QZS0, Q9CY78, Q9D155, Q9JLZ3

    3. NM_001426112.1 → NP_001413041.1  methylglutaconyl-CoA hydratase, mitochondrial isoform 4

      Status: VALIDATED

      Source sequence(s)
      AC140443, AC160765
      UniProtKB/Swiss-Prot
      Q80YD7, Q8QZS0, Q9CY78, Q9D155, Q9JLZ3

    4. NM_001426113.1 → NP_001413042.1  methylglutaconyl-CoA hydratase, mitochondrial isoform 5

      Status: VALIDATED

      Source sequence(s)
      AC140443, AC160765
      UniProtKB/Swiss-Prot
      Q80YD7, Q8QZS0, Q9CY78, Q9D155, Q9JLZ3

    5. NM_001426114.1 → NP_001413043.1  methylglutaconyl-CoA hydratase, mitochondrial isoform 6

      Status: VALIDATED

      Source sequence(s)
      AC140443, AC160765
      UniProtKB/Swiss-Prot
      Q80YD7, Q8QZS0, Q9CY78, Q9D155, Q9JLZ3

    6. NM_001426115.1 → NP_001413044.1  methylglutaconyl-CoA hydratase, mitochondrial isoform 7

      Status: VALIDATED

      Source sequence(s)
      AC140443, AC160765
      UniProtKB/Swiss-Prot
      Q80YD7, Q8QZS0, Q9CY78, Q9D155, Q9JLZ3

    7. NM_001426116.1 → NP_001413045.1  methylglutaconyl-CoA hydratase, mitochondrial isoform 8

      Status: VALIDATED

      Source sequence(s)
      AC140443, AC160765
      UniProtKB/Swiss-Prot
      Q80YD7, Q8QZS0, Q9CY78, Q9D155, Q9JLZ3

    8. NM_001426117.1 → NP_001413046.1  methylglutaconyl-CoA hydratase, mitochondrial isoform 9

      Status: VALIDATED

      Source sequence(s)
      AC140443, AC160765
      UniProtKB/Swiss-Prot
      Q80YD7, Q8QZS0, Q9CY78, Q9D155, Q9JLZ3

    9. NM_001426118.1 → NP_001413047.1  methylglutaconyl-CoA hydratase, mitochondrial isoform 10

      Status: VALIDATED

      Source sequence(s)
      AC140443, AC160765
      UniProtKB/Swiss-Prot
      Q80YD7, Q8QZS0, Q9CY78, Q9D155, Q9JLZ3

    10. NM_001426119.1 → NP_001413048.1  methylglutaconyl-CoA hydratase, mitochondrial isoform 11

      Status: VALIDATED

      Source sequence(s)
      AC140443, AC160765

    11. NM_001426120.1 → NP_001413049.1  methylglutaconyl-CoA hydratase, mitochondrial isoform 12

      Status: VALIDATED

      Source sequence(s)
      AC140443, AC160765

    12. NM_001426121.1 → NP_001413050.1  methylglutaconyl-CoA hydratase, mitochondrial isoform 13

      Status: VALIDATED

      Source sequence(s)
      AC140443, AC160765

    13. NM_001426122.1 → NP_001413051.1  methylglutaconyl-CoA hydratase, mitochondrial isoform 13

      Status: VALIDATED

      Source sequence(s)
      AC140443, AC160765

    14. NM_016709.3 → NP_057918.2  methylglutaconyl-CoA hydratase, mitochondrial isoform 1

      See identical proteins and their annotated locations for NP_057918.2

      Status: VALIDATED

      Source sequence(s)
      AC140443, AC160765
      Consensus CDS
      CCDS26518.1
      UniProtKB/Swiss-Prot
      Q80YD7, Q8QZS0, Q9CY78, Q9D155, Q9JLZ3
      UniProtKB/TrEMBL
      A0A0R4J023
      Related
      ENSMUSP00000021913.10, ENSMUST00000021913.16
      Conserved Domains (1) summary
      cl23717
      Location:61 → 314
      crotonase-like; Crotonase/Enoyl-Coenzyme A (CoA) hydratase superfamily. This superfamily contains a diverse set of enzymes including enoyl-CoA hydratase, napthoate synthase, methylmalonyl-CoA decarboxylase, 3-hydoxybutyryl-CoA dehydratase, and dienoyl-CoA isomerase. ...

    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_000079.7 Reference GRCm39 C57BL/6J

      Range
      52989155..53083742 complement
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. XM_017315373.3 → XP_017170862.1  methylglutaconyl-CoA hydratase, mitochondrial isoform X2

      UniProtKB/TrEMBL
      E9QMT1
      Conserved Domains (1) summary
      cl23717
      Location:61 → 114
      crotonase-like; Crotonase/Enoyl-Coenzyme A (CoA) hydratase superfamily. This superfamily contains a diverse set of enzymes including enoyl-CoA hydratase, napthoate synthase, methylmalonyl-CoA decarboxylase, 3-hydoxybutyryl-CoA dehydratase, and dienoyl-CoA isomerase. ...

    2. XM_006516846.5 → XP_006516909.1  methylglutaconyl-CoA hydratase, mitochondrial isoform X2

      UniProtKB/TrEMBL
      E9QMT1
      Related
      ENSMUSP00000105658.4, ENSMUST00000110031.4
      Conserved Domains (1) summary
      cl23717
      Location:61 → 114
      crotonase-like; Crotonase/Enoyl-Coenzyme A (CoA) hydratase superfamily. This superfamily contains a diverse set of enzymes including enoyl-CoA hydratase, napthoate synthase, methylmalonyl-CoA decarboxylase, 3-hydoxybutyryl-CoA dehydratase, and dienoyl-CoA isomerase. ...

    RNA

    1. XR_003950422.2 RNA Sequence

    Nucleotide Protein Strain
    Heading Accession and Version
    Protein Accession Links
    GenPept Link UniProtKB Link
    Q9JLZ3.1 GenPept UniProtKB/Swiss-Prot:Q9JLZ3

    Gene LinkOut

    The following LinkOut resources are supplied by external providers. These providers are responsible for maintaining the links.

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