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Hadhb hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit beta [ Rattus norvegicus (Norway rat) ]

Gene ID: 171155, updated on 13-Apr-2024

Summary

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Official Symbol
Hadhbprovided by RGD
Official Full Name
hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit betaprovided by RGD
Primary source
RGD:620513
See related
Ensembl:ENSRNOG00000010800 AllianceGenome:RGD:620513
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
Also known as
TP-beta
Summary
Enables acetyl-CoA C-acyltransferase activity. Involved in fatty acid beta-oxidation. Located in mitochondrion. Part of mitochondrial fatty acid beta-oxidation multienzyme complex. Human ortholog(s) of this gene implicated in mitochondrial metabolism disease and mitochondrial trifunctional protein deficiency. Orthologous to human HADHB (hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit beta). [provided by Alliance of Genome Resources, Apr 2022]
Expression
Biased expression in Heart (RPKM 3658.8), Muscle (RPKM 1788.8) and 8 other tissues See more
Orthologs
human mouse all
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Genomic context

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Location:
6q14
Exon count:
18
Annotation release Status Assembly Chr Location
RS_2024_02 current GRCr8 (GCF_036323735.1) 6 NC_086024.1 (31873404..31907557, complement)
RS_2023_06 previous assembly mRatBN7.2 (GCF_015227675.2) 6 NC_051341.1 (26153572..26187668, complement)
106 previous assembly Rnor_6.0 (GCF_000001895.5) 6 NC_005105.4 (27555408..27589539, complement)

Chromosome 6 - NC_086024.1Genomic Context describing neighboring genes Neighboring gene uncharacterized LOC120103489 Neighboring gene adhesion G protein-coupled receptor F3 Neighboring gene hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha Neighboring gene GRB2 associated regulator of MAPK1 subtype 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. Characterization of mitochondrial trifunctional protein and its inactivation study for medicine development. Liu X, et al. Biochim Biophys Acta, 2008 Nov. PMID 18640292
  2. ENU mutagenesis identifies mice with cardiac fibrosis and hepatic steatosis caused by a mutation in the mitochondrial trifunctional protein beta-subunit. Kao HJ, et al. Hum Mol Genet, 2006 Dec 15. PMID 17116638
  3. The mitochondrial trifunctional protein: centre of a beta-oxidation metabolon? Eaton S, et al. Biochem Soc Trans, 2000 Feb. PMID 10816122
  4. Molecular characterization of mitochondrial trifunctional protein deficiency: formation of the enzyme complex is important for stabilization of both alpha- and beta-subunits. Ushikubo S, et al. Am J Hum Genet, 1996 May. PMID 8651282, Free PMC Article
  5. Molecular cloning of the cDNAs for the subunits of rat mitochondrial fatty acid beta-oxidation multienzyme complex. Structural and functional relationships to other mitochondrial and peroxisomal beta-oxidation enzymes. Kamijo T, et al. J Biol Chem, 1993 Dec 15. PMID 8253773

See all (24) citations in PubMed

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
  1. The catalytic residues of the MTP beta-subunit are positioned in the active site similarly to those of monofunctional 3-ketoacyl-CoA thiolase.
    Title: Characterization of mitochondrial trifunctional protein and its inactivation study for medicine development.
Submit: New GeneRIF Correction

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 RGD

Function Evidence Code Pubs
enables acetyl-CoA C-acetyltransferase activity IEA
Inferred from Electronic Annotation
more info
  
enables acetyl-CoA C-acetyltransferase activity ISO
Inferred from Sequence Orthology
more info
  
enables acetyl-CoA C-acyltransferase activity IDA
Inferred from Direct Assay
more info
 PubMed 
enables acetyl-CoA C-acyltransferase activity ISO
Inferred from Sequence Orthology
more info
  
enables acetyl-CoA C-myristoyltransferase activity IEA
Inferred from Electronic Annotation
more info
  
enables lncRNA binding IEA
Inferred from Electronic Annotation
more info
  
enables lncRNA binding ISO
Inferred from Sequence Orthology
more info
  
enables protein-containing complex binding IMP
Inferred from Mutant Phenotype
more info
 PubMed 
Process Evidence Code Pubs
involved_in cellular response to lipopolysaccharide IEA
Inferred from Electronic Annotation
more info
  
involved_in cellular response to lipopolysaccharide ISO
Inferred from Sequence Orthology
more info
  
involved_in fatty acid beta-oxidation IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in fatty acid beta-oxidation IDA
Inferred from Direct Assay
more info
 PubMed 
involved_in fatty acid beta-oxidation IEA
Inferred from Electronic Annotation
more info
  
involved_in fatty acid beta-oxidation ISO
Inferred from Sequence Orthology
more info
  
involved_in gene expression IEA
Inferred from Electronic Annotation
more info
  
involved_in gene expression ISO
Inferred from Sequence Orthology
more info
  
Component Evidence Code Pubs
located_in endoplasmic reticulum IEA
Inferred from Electronic Annotation
more info
  
located_in endoplasmic reticulum ISO
Inferred from Sequence Orthology
more info
  
located_in endoplasmic reticulum ISS
Inferred from Sequence or Structural Similarity
more info
  
part_of mitochondrial fatty acid beta-oxidation multienzyme complex IDA
Inferred from Direct Assay
more info
 PubMed 
part_of mitochondrial fatty acid beta-oxidation multienzyme complex IEA
Inferred from Electronic Annotation
more info
  
part_of mitochondrial fatty acid beta-oxidation multienzyme complex ISO
Inferred from Sequence Orthology
more info
  
located_in mitochondrial inner membrane IEA
Inferred from Electronic Annotation
more info
  
located_in mitochondrial inner membrane ISO
Inferred from Sequence Orthology
more info
  
located_in mitochondrial inner membrane ISS
Inferred from Sequence or Structural Similarity
more info
  
located_in mitochondrial nucleoid IEA
Inferred from Electronic Annotation
more info
  
located_in mitochondrial nucleoid ISO
Inferred from Sequence Orthology
more info
  
located_in mitochondrial outer membrane IEA
Inferred from Electronic Annotation
more info
  
located_in mitochondrial outer membrane ISO
Inferred from Sequence Orthology
more info
  
located_in mitochondrial outer membrane ISS
Inferred from Sequence or Structural Similarity
more info
  
is_active_in mitochondrion IBA
Inferred from Biological aspect of Ancestor
more info
  
is_active_in mitochondrion ISO
Inferred from Sequence Orthology
more info
  
located_in mitochondrion ISO
Inferred from Sequence Orthology
more info
  

General protein information

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Preferred Names
trifunctional enzyme subunit beta, mitochondrial
Names
hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit
hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit
mitochondrial trifunctional protein, beta subunit
NP_598302.1
XP_006239848.2
XP_038967674.1
XP_063117589.1
XP_063117590.1
XP_063117591.1
XP_063117592.1
XP_063117593.1

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_133618.3 → NP_598302.1  trifunctional enzyme subunit beta, mitochondrial precursor

    See identical proteins and their annotated locations for NP_598302.1

    Status: VALIDATED

    Source sequence(s)
    FM038662, FM050540, FM105991, FM118580, JAXUCZ010000006
    UniProtKB/Swiss-Prot
    Q60587
    UniProtKB/TrEMBL
    A0A0G2K330, A6HAE3
    Conserved Domains (1) summary
    cd00751
    Location:56 → 472
    thiolase; Thiolase are ubiquitous enzymes that catalyze the reversible thiolytic cleavage of 3-ketoacyl-CoA into acyl-CoA and acetyl-CoA, a 2-step reaction involving a covalent intermediate formed with a catalytic cysteine. They are found in prokaryotes and ...

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_086024.1 Reference GRCr8

    Range
    31873404..31907557 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_063261523.1 → XP_063117593.1  trifunctional enzyme subunit beta, mitochondrial isoform X1

  2. XM_039111746.2 → XP_038967674.1  trifunctional enzyme subunit beta, mitochondrial isoform X2

    UniProtKB/Swiss-Prot
    Q60587
    UniProtKB/TrEMBL
    A0A0G2K330, A6HAE3
    Conserved Domains (1) summary
    cd00751
    Location:56 → 472
    thiolase; Thiolase are ubiquitous enzymes that catalyze the reversible thiolytic cleavage of 3-ketoacyl-CoA into acyl-CoA and acetyl-CoA, a 2-step reaction involving a covalent intermediate formed with a catalytic cysteine. They are found in prokaryotes and ...

  3. XM_063261520.1 → XP_063117590.1  trifunctional enzyme subunit beta, mitochondrial isoform X1

  4. XM_063261522.1 → XP_063117592.1  trifunctional enzyme subunit beta, mitochondrial isoform X1

  5. XM_006239786.4 → XP_006239848.2  trifunctional enzyme subunit beta, mitochondrial isoform X2

    UniProtKB/Swiss-Prot
    Q60587
    UniProtKB/TrEMBL
    A0A0G2K330, A6HAE3
    Conserved Domains (1) summary
    cd00751
    Location:56 → 472
    thiolase; Thiolase are ubiquitous enzymes that catalyze the reversible thiolytic cleavage of 3-ketoacyl-CoA into acyl-CoA and acetyl-CoA, a 2-step reaction involving a covalent intermediate formed with a catalytic cysteine. They are found in prokaryotes and ...

  6. XM_063261519.1 → XP_063117589.1  trifunctional enzyme subunit beta, mitochondrial isoform X1

  7. XM_063261521.1 → XP_063117591.1  trifunctional enzyme subunit beta, mitochondrial isoform X1

Nucleotide Protein Strain
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
Q60587.1 GenPept UniProtKB/Swiss-Prot:Q60587

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