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

Gene ID: 361215, updated on 11-Apr-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
Ensembl: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 and mRNA 3'-UTR binding 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, Apr 2022]
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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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 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 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
    Related
    ENSRNOP00000015786.5, ENSRNOT00000015786.8
    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, ENSRNOT00000096716.1
    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
    Related
    ENSRNOP00000095770.1
    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

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

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