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    Hadh hydroxyacyl-Coenzyme A dehydrogenase [ Mus musculus (house mouse) ]

    Gene ID: 15107, updated on 5-Mar-2024

    Summary

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    Official Symbol
    Hadhprovided by MGI
    Official Full Name
    hydroxyacyl-Coenzyme A dehydrogenaseprovided by MGI
    Primary source
    MGI:MGI:96009
    See related
    Ensembl:ENSMUSG00000027984 AllianceGenome:MGI:96009
    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
    HCDH; Schad; Hadhsc
    Summary
    Enables 3-hydroxyacyl-CoA dehydrogenase activity. Involved in positive regulation of cold-induced thermogenesis and regulation of insulin secretion. Located in mitochondrion. Is expressed in several structures, including alimentary system; brain; genitourinary system; respiratory system; and sensory organ. Human ortholog(s) of this gene implicated in familial hyperinsulinemic hypoglycemia 4. Orthologous to human HADH (hydroxyacyl-CoA dehydrogenase). [provided by Alliance of Genome Resources, Apr 2022]
    Expression
    Broad expression in heart adult (RPKM 232.8), kidney adult (RPKM 182.9) and 21 other tissues See more
    Orthologs
    human all
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    Genomic context

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    Location:
    3 G3; 3 60.86 cM
    Exon count:
    8
    Annotation release Status Assembly Chr Location
    RS_2024_02 current GRCm39 (GCF_000001635.27) 3 NC_000069.7 (131027068..131065750, complement)
    108.20200622 previous assembly GRCm38.p6 (GCF_000001635.26) 3 NC_000069.6 (131233419..131272101, complement)

    Chromosome 3 - NC_000069.7Genomic Context describing neighboring genes Neighboring gene STARR-seq mESC enhancer starr_08995 Neighboring gene predicted gene, 36603 Neighboring gene STARR-positive B cell enhancer ABC_E6127 Neighboring gene lymphoid enhancer binding factor 1 Neighboring gene CapStarr-seq enhancer MGSCv37_chr3:130860450-130860637 Neighboring gene CapStarr-seq enhancer MGSCv37_chr3:130911499-130911865 Neighboring gene STARR-positive B cell enhancer ABC_E6128 Neighboring gene TatD DNase domain containing 2 pseudogene Neighboring gene STARR-positive B cell enhancer ABC_E11712 Neighboring gene cytochrome P450, family 2, subfamily u, polypeptide 1

    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. The Hypoglycemic Phenotype Is Islet Cell-Autonomous in Short-Chain Hydroxyacyl-CoA Dehydrogenase-Deficient Mice. Molven A, et al. Diabetes, 2016 Jun. PMID 26953163, Free PMC Article
    2. Functional genomics of the beta-cell: short-chain 3-hydroxyacyl-coenzyme A dehydrogenase regulates insulin secretion independent of K+ currents. Hardy OT, et al. Mol Endocrinol, 2007 Mar. PMID 17185391
    3. L-3-hydroxyacyl-CoA dehydrogenase II protects in a model of Parkinson's disease. Tieu K, et al. Ann Neurol, 2004 Jul. PMID 15236401
    4. Isolation of a cDNA clone encoding mouse 3-hydroxyacyl CoA dehydrogenase. Nomura M, et al. Gene, 1995 Jul 28. PMID 7642117
    5. Hadh deficiency induced oligoasthenoteratozoospermia through the TNF-α/Bcl-2 pathway in male mice. Zhu H, et al. FASEB J, 2022 Dec. PMID 36398584

    See all (46) citations in PubMed

    GeneRIFs: Gene References Into Functions

    What's a GeneRIF?
    1. Hadh deficiency induced oligoasthenoteratozoospermia through the TNF-alpha/Bcl-2 pathway in male mice.
      Title: Hadh deficiency induced oligoasthenoteratozoospermia through the TNF-α/Bcl-2 pathway in male mice.
    2. A physical association between short-chain 3-hydroxyacyl-coenzyme A dehydrogenase and important components of other key metabolic pathways. Most of the interactions were with enzymes in mitochondrial pathways.
      Title: Short-chain 3-hydroxyacyl-coenzyme A dehydrogenase associates with a protein super-complex integrating multiple metabolic pathways.
    3. SCHAD is involved in thermogenesis, in the maintenance of body weight, and in the regulation of nutrient-stimulated insulin secretion
      Title: Role of medium- and short-chain L-3-hydroxyacyl-CoA dehydrogenase in the regulation of body weight and thermogenesis.
    4. SCHAD deficiency causes hyperinsulinism by activation of GDH via loss of inhibitory regulation of GDH by SCHAD.
      Title: Mechanism of hyperinsulinism in short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency involves activation of glutamate dehydrogenase.
    5. SCHAD regulates insulin secretion through a KATP channel-independent mechanism.
      Title: Functional genomics of the beta-cell: short-chain 3-hydroxyacyl-coenzyme A dehydrogenase regulates insulin secretion independent of K+ currents.
    6. Results demonstrate that L-3-hydroxyacyl-CoA dehydrogenase type II (HADH II/ABAD) modulates 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine neurotoxicity and suggests that mimetics may provide protective benefit in the treatment of Parkinson disease.
      Title: L-3-hydroxyacyl-CoA dehydrogenase II protects in a model of Parkinson's disease.
    Submit: New GeneRIF Correction

    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 3-hydroxyacyl-CoA dehydrogenase activity IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    enables 3-hydroxyacyl-CoA dehydrogenase activity IDA
    Inferred from Direct Assay
    more info
    		 PubMed 
    enables 3-hydroxyacyl-CoA dehydrogenase activity ISO
    Inferred from Sequence Orthology
    more info
    		  
    enables NAD+ binding ISO
    Inferred from Sequence Orthology
    more info
    		  
    enables identical protein binding ISO
    Inferred from Sequence Orthology
    more info
    		  
    enables oxidoreductase activity IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables protein binding IPI
    Inferred from Physical Interaction
    more info
    		 PubMed 
    Process Evidence Code Pubs
    involved_in fatty acid beta-oxidation IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    involved_in fatty acid beta-oxidation ISO
    Inferred from Sequence Orthology
    more info
    		  
    acts_upstream_of_or_within fatty acid metabolic process IEA
    Inferred from Electronic Annotation
    more info
    		  
    involved_in fatty acid metabolic process IEA
    Inferred from Electronic Annotation
    more info
    		  
    acts_upstream_of_or_within lipid metabolic process IEA
    Inferred from Electronic Annotation
    more info
    		  
    involved_in negative regulation of insulin secretion ISO
    Inferred from Sequence Orthology
    more info
    		  
    involved_in positive regulation of cold-induced thermogenesis IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    involved_in regulation of insulin secretion IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    involved_in response to activity ISO
    Inferred from Sequence Orthology
    more info
    		  
    involved_in response to xenobiotic stimulus ISO
    Inferred from Sequence Orthology
    more info
    		  
    Component Evidence Code Pubs
    located_in cytoplasm ISO
    Inferred from Sequence Orthology
    more info
    		  
    located_in mitochondrial inner membrane HDA PubMed 
    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 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
    hydroxyacyl-coenzyme A dehydrogenase, mitochondrial
    Names
    L-3-hydroxyacyl-Coenzyme A dehydrogenase, short chain
    hydroxylacyl-Coenzyme A dehydrogenase-dehydrogenase
    medium and short chain L-3-hydroxyacyl-coenzyme A dehydrogenase
    short-chain 3-hydroxyacyl-CoA dehydrogenase
    NP_032238.2

    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_008212.4 → NP_032238.2  hydroxyacyl-coenzyme A dehydrogenase, mitochondrial precursor

      See identical proteins and their annotated locations for NP_032238.2

      Status: VALIDATED

      Source sequence(s)
      AC123634, AI845880, AK132260, AK148486
      Consensus CDS
      CCDS38640.1
      UniProtKB/Swiss-Prot
      Q3TF75, Q3THK8, Q3UFI0, Q61425, Q8K149, Q925U9
      Related
      ENSMUSP00000029610.9, ENSMUST00000029610.9
      Conserved Domains (3) summary
      COG1250
      Location:27 → 313
      FadB; 3-hydroxyacyl-CoA dehydrogenase [Lipid transport and metabolism]
      pfam00725
      Location:216 → 313
      3HCDH; 3-hydroxyacyl-CoA dehydrogenase, C-terminal domain
      pfam02737
      Location:29 → 214
      3HCDH_N; 3-hydroxyacyl-CoA dehydrogenase, NAD binding domain

    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_000069.7 Reference GRCm39 C57BL/6J

      Range
      131027068..131065750 complement
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)
    Nucleotide Protein Strain
    Heading Accession and Version
    Protein Accession Links
    GenPept Link UniProtKB Link
    Q61425.2 GenPept UniProtKB/Swiss-Prot:Q61425

    Gene LinkOut

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

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