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

Summary

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

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_086024.1 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Genomic Sequence:

Go to nucleotide: Graphics FASTA GenBank

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

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

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

RH134632 (e-PCR)
- UniSTS:217911

D6Got20 (e-PCR)
- UniSTS:113561

BE100255 (e-PCR)
- UniSTS:249793

Hadhb (e-PCR), detects polymorphism
- UniSTS:546814

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	IEA Inferred from Electronic Annotation more info
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

EC 2.3.1.155

EC 2.3.1.16

XP_006239848.2

EC 2.3.1.155

EC 2.3.1.16

XP_038967674.1

EC 2.3.1.155

EC 2.3.1.16

XP_063117589.1

EC 2.3.1.155

EC 2.3.1.16

XP_063117590.1

EC 2.3.1.155

EC 2.3.1.16

XP_063117591.1

EC 2.3.1.155

EC 2.3.1.16

XP_063117592.1

EC 2.3.1.155

EC 2.3.1.16

XP_063117593.1

EC 2.3.1.155

EC 2.3.1.16

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)

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

NC_086024.1 Reference GRCr8

Range

31873404..31907557 complement

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GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

XM_063261523.1 → XP_063117593.1 trifunctional enzyme subunit beta, mitochondrial isoform X1
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 ...
XM_063261520.1 → XP_063117590.1 trifunctional enzyme subunit beta, mitochondrial isoform X1
XM_063261522.1 → XP_063117592.1 trifunctional enzyme subunit beta, mitochondrial isoform X1
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 ...
XM_063261519.1 → XP_063117589.1 trifunctional enzyme subunit beta, mitochondrial isoform X1
XM_063261521.1 → XP_063117591.1 trifunctional enzyme subunit beta, mitochondrial isoform X1