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    Auh AU RNA binding methylglutaconyl-CoA hydratase [ Rattus norvegicus (Norway rat) ]

    Gene ID: 361215, updated on 9-Dec-2024

    Summary

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    Official Symbol
    Auhprovided by RGD
    Official Full Name
    AU RNA binding methylglutaconyl-CoA hydrataseprovided by RGD
    Primary source
    RGD:1306087
    See related
    EnsemblRapid:ENSRNOG00000011684 AllianceGenome:RGD:1306087
    Gene type
    protein coding
    RefSeq status
    VALIDATED
    Organism
    Rattus norvegicus
    Lineage
    Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Rattus
    Summary
    Predicted to enable enoyl-CoA hydratase activity; mRNA 3'-UTR binding activity; and methylglutaconyl-CoA hydratase activity. Predicted to be involved in fatty acid beta-oxidation. Predicted to be active 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
    Biased expression in Adrenal (RPKM 239.2), Kidney (RPKM 164.8) and 9 other tissues See more
    Orthologs
    human mouse all
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    Genomic context

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    See Auh in Genome Data Viewer
    Location:
    17p14
    Exon count:
    11
    Annotation release Status Assembly Chr Location
    RS_2024_02 current GRCr8 (GCF_036323735.1) 17 NC_086035.1 (12481396..12576748)
    RS_2023_06 previous assembly mRatBN7.2 (GCF_015227675.2) 17 NC_051352.1 (12329522..12424882)
    106 previous assembly Rnor_6.0 (GCF_000001895.5) 17 NC_005116.4 (12310178..12405224)

    Chromosome 17 - NC_086035.1Genomic Context describing neighboring genes Neighboring gene uncharacterized LOC120097877 Neighboring gene nuclear factor, interleukin 3 regulated Neighboring gene uncharacterized LOC134482816 Neighboring gene uncharacterized LOC120097808 Neighboring gene uncharacterized LOC108348512 Neighboring gene ribosomal protein S28, pseudogene 2

    Genomic regions, transcripts, and products

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    Go to nucleotide: Graphics FASTA GenBank

    Expression

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    • Project title: A rat RNA-Seq transcriptomic BodyMap across 11 organs and 4 developmental stages
    • Description: 320 RNA samples isolated from 11 organs (adrenal gland, brain, heart, kidney, liver, lung, muscle, spleen, thymus, and testes or uterus) from both sexes of Fischer 344 rats across four developmental stages (2-, 6-, 21-, and 104-weeks-old)
    • BioProject: PRJNA238328
    • Publication: PMID 24510058
    • Analysis date: Mon Jun 6 17:44:12 2016

    Bibliography

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    Related articles in PubMed

    1. 3-Methylglutaconic aciduria type I is caused by mutations in AUH. IJlst L, et al. Am J Hum Genet, 2002 Dec. PMID 12434311, Free PMC Article
    2. AUH, a gene encoding an AU-specific RNA binding protein with intrinsic enoyl-CoA hydratase activity. Nakagawa J, et al. Proc Natl Acad Sci U S A, 1995 Mar 14. PMID 7892223, Free PMC Article
    3. Characterisation and mitochondrial localisation of AUH, an AU-specific RNA-binding enoyl-CoA hydratase. Brennan LE, et al. Gene, 1999 Mar 4. PMID 10072761
    4. Integrated analysis of protein composition, tissue diversity, and gene regulation in mouse mitochondria. Mootha VK, et al. Cell, 2003 Nov 26. PMID 14651853
    5. A mitochondrial protein compendium elucidates complex I disease biology. Pagliarini DJ, et al. Cell, 2008 Jul 11. PMID 18614015, Free PMC Article

    See all (7) citations in PubMed

    GeneRIFs: Gene References Into Functions

    Pathways from PubChem

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    General gene information

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    Markers

    Homology

    Gene Ontology Provided by RGD

    Function Evidence Code Pubs
    enables RNA binding ISO
    Inferred from Sequence Orthology
    more info
    		  
    enables catalytic activity IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables enoyl-CoA hydratase activity IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    enables enoyl-CoA hydratase activity ISO
    Inferred from Sequence Orthology
    more info
    		  
    enables itaconyl-CoA hydratase activity IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables lyase activity IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables mRNA 3'-UTR binding ISO
    Inferred from Sequence Orthology
    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 biological_process ND
    No biological Data available
    more info
    		  
    involved_in fatty acid beta-oxidation IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    Component Evidence Code Pubs
    is_active_in mitochondrion IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    located_in mitochondrion ISO
    Inferred from Sequence Orthology
    more info
    		  

    General protein information

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    Preferred Names
    methylglutaconyl-CoA hydratase, mitochondrial
    Names
    3-MG-CoA hydratase
    AU RNA binding protein/enoyl-CoA hydratase
    AU RNA binding protein/enoyl-coenzyme A hydratase
    AU-binding enoyl-CoA hydratase
    AU-specific RNA-binding enoyl-CoA hydratase
    itaconyl-CoA hydratase
    NP_001386179.1
    XP_006253737.1
    XP_008769721.1
    XP_038951784.1

    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_001399250.1 → NP_001386179.1  methylglutaconyl-CoA hydratase, mitochondrial

      Status: VALIDATED

      Source sequence(s)
      JAXUCZ010000017
      UniProtKB/Swiss-Prot
      F1LU71
      UniProtKB/TrEMBL
      A0A8I5Y7Q4
      Related
      ENSRNOP00000015786.5, ENSRNOT00000015786.9
      Conserved Domains (1) summary
      cl23717
      Location:62 → 315
      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_036323735.1-RS_2024_02

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

    Reference GRCr8

    Genomic

    1. NC_086035.1 Reference GRCr8

      Range
      12481396..12576748
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. XM_039095856.2 → XP_038951784.1  methylglutaconyl-CoA hydratase, mitochondrial isoform X3

      UniProtKB/TrEMBL
      A6J6R7
      Conserved Domains (1) summary
      cl23717
      Location:64 → 184
      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_006253675.3 → XP_006253737.1  methylglutaconyl-CoA hydratase, mitochondrial isoform X1

      UniProtKB/TrEMBL
      A0A8I5Y7Q4, A0A8I6AAT9
      Related
      ENSRNOP00000090436.1, ENSRNOT00000096716.2
      Conserved Domains (1) summary
      cl23717
      Location:62 → 284
      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. ...

    3. XM_008771499.4 → XP_008769721.1  methylglutaconyl-CoA hydratase, mitochondrial isoform X2

      UniProtKB/TrEMBL
      A0A8I6B3A4, A6J6R8
      Conserved Domains (1) summary
      cl23717
      Location:2 → 230
      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_005495294.2 RNA Sequence

    2. XR_005495293.2 RNA Sequence

      Related
      ENSRNOT00000167904.1

    Suppressed Reference Sequence(s)

    The following Reference Sequences have been suppressed. Explain

    1. NM_001108407.1: Suppressed sequence

      Description
      NM_001108407.1: This RefSeq was removed because currently there is insufficient support for the transcript.
    Nucleotide Protein Strain
    Heading Accession and Version
    Protein Accession Links
    GenPept Link UniProtKB Link
    F1LU71.2 GenPept UniProtKB/Swiss-Prot:F1LU71

    Gene LinkOut

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

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