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

Summary

Official Symbol: Hadhaprovided by RGD
Official Full Name: hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alphaprovided by RGD
Primary source: RGD:620512
See related: EnsemblRapid:ENSRNOG00000024629 AllianceGenome:RGD:620512
Gene type: protein coding
RefSeq status: PROVISIONAL
Organism: Rattus norvegicus
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Rattus
Also known as: RGD1560655
Summary: Enables several functions, including 3-hydroxyacyl-CoA dehydratase activity; long-chain-3-hydroxyacyl-CoA dehydrogenase activity; and nucleoside phosphate binding activity. Involved in fatty acid beta-oxidation and response to xenobiotic stimulus. Part of mitochondrial fatty acid beta-oxidation multienzyme complex. Biomarker of metabolic dysfunction-associated steatotic liver disease. Human ortholog(s) of this gene implicated in mitochondrial trifunctional protein deficiency and steatotic liver disease. Orthologous to human HADHA (hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha). [provided by Alliance of Genome Resources, Dec 2024]
Expression: Biased expression in Heart (RPKM 2489.0), Adrenal (RPKM 1634.2) and 9 other tissues See more
Orthologs: human mouse all
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Genomic context

Location:: 6q14

Exon count:: 20

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCr8 (GCF_036323735.1)	6	NC_086024.1 (31907801..31947434)
RS_2023_06	previous assembly	mRatBN7.2 (GCF_015227675.2)	6	NC_051341.1 (26187969..26227605)
106	previous assembly	Rnor_6.0 (GCF_000001895.5)	6	NC_005105.4 (27589840..27628921)

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

Expression

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See details

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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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

AI535136 (e-PCR)
- UniSTS:248085

RH128498 (e-PCR)
- UniSTS:211806

RH143343 (e-PCR)
- UniSTS:232590

D12S1229E (e-PCR)
- UniSTS:151476

BE100255 (e-PCR)
- UniSTS:249793

Gene Ontology Provided by RGD

Function	Evidence Code	Pubs
enables 3-hydroxyacyl-CoA dehydratase activity	IDA Inferred from Direct Assay more info	PubMed
enables 3-hydroxyacyl-CoA dehydrogenase activity	IDA Inferred from Direct Assay more info	PubMed
enables 3-hydroxyacyl-CoA dehydrogenase activity	IEA Inferred from Electronic Annotation more info
enables 3-hydroxyacyl-CoA dehydrogenase activity	IMP Inferred from Mutant Phenotype more info	PubMed
enables NAD binding	IDA Inferred from Direct Assay more info	PubMed
enables NAD+ binding	IEA Inferred from Electronic Annotation more info
enables acetyl-CoA C-acyltransferase activity	IDA Inferred from Direct Assay more info	PubMed
enables enoyl-CoA hydratase activity	IBA Inferred from Biological aspect of Ancestor more info
enables enoyl-CoA hydratase activity	IDA Inferred from Direct Assay more info	PubMed
enables enoyl-CoA hydratase activity	IMP Inferred from Mutant Phenotype more info	PubMed
enables fatty-acyl-CoA binding	IDA Inferred from Direct Assay more info	PubMed
enables long-chain-3-hydroxyacyl-CoA dehydrogenase activity	IBA Inferred from Biological aspect of Ancestor more info
enables long-chain-3-hydroxyacyl-CoA dehydrogenase activity	IDA Inferred from Direct Assay more info	PubMed
enables long-chain-3-hydroxyacyl-CoA dehydrogenase activity	ISO Inferred from Sequence Orthology more info
enables lyase activity	IEA Inferred from Electronic Annotation more info
enables protein-containing complex binding	IMP Inferred from Mutant Phenotype more info	PubMed

Process	Evidence Code	Pubs
involved_in cardiolipin acyl-chain remodeling	ISO Inferred from Sequence Orthology more info
involved_in cardiolipin acyl-chain remodeling	ISS Inferred from Sequence or Structural Similarity 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
acts_upstream_of_or_within fatty acid beta-oxidation	ISO Inferred from Sequence Orthology more info
involved_in fatty acid beta-oxidation	ISO Inferred from Sequence Orthology more info
acts_upstream_of_or_within response to insulin	ISO Inferred from Sequence Orthology more info
involved_in response to xenobiotic stimulus	IDA Inferred from Direct Assay more info	PubMed

Component	Evidence Code	Pubs
part_of mitochondrial fatty acid beta-oxidation multienzyme complex	IBA Inferred from Biological aspect of Ancestor 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	ISO Inferred from Sequence Orthology more info
part_of mitochondrial fatty acid beta-oxidation multienzyme complex	TAS Traceable Author Statement more info	PubMed
located_in mitochondrial inner membrane	ISO Inferred from Sequence Orthology more info
located_in mitochondrial nucleoid	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 alpha, mitochondrial

Names: TP-alpha; hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit; hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit; monolysocardiolipin acyltransferase

NP_570839.2

EC 1.1.1.211

EC 4.2.1.17

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_130826.2 → NP_570839.2 trifunctional enzyme subunit alpha, mitochondrial precursor

See identical proteins and their annotated locations for NP_570839.2

Status: PROVISIONAL

Source sequence(s)

BC091697

UniProtKB/Swiss-Prot

Q5BIZ5, Q64428

UniProtKB/TrEMBL

A0A8I6A8P4, A6HAE4

Related

ENSRNOP00000107572.1, ENSRNOT00000142694.1

Conserved Domains (1) summary

TIGR02441
Location:29 → 762

fa_ox_alpha_mit; fatty acid oxidation complex, alpha subunit, mitochondrial