Hadha hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: Hadhaprovided by MGI
Official Full Name: hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alphaprovided by MGI
Primary source: MGI:MGI:2135593
See related: Ensembl:ENSMUSG00000025745 AllianceGenome:MGI:2135593
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: Mtpa; TP-alpha
Summary: Enables long-chain-3-hydroxyacyl-CoA dehydrogenase activity. Acts upstream of or within fatty acid beta-oxidation and response to insulin. Located in mitochondrial inner membrane. Is expressed in several structures, including alimentary system; genitourinary system; nervous system; respiratory system; and sensory organ. Human ortholog(s) of this gene implicated in mitochondrial trifunctional protein deficiency 1 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: Ubiquitous expression in heart adult (RPKM 149.0), liver E18 (RPKM 62.5) and 28 other tissues See more
Orthologs: human all
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Genomic context

Location:: 5 B1; 5 15.95 cM

Exon count:: 20

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	5	NC_000071.7 (30324421..30359978, complement)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	5	NC_000071.6 (30119423..30154980, complement)

Chromosome 5 - NC_000071.7 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Genomic Sequence:

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Expression

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See details

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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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

Zfp335 establishes eTreg lineage and neonatal immune tolerance by targeting Hadha-mediated fatty acid oxidation.
Title: Zfp335 establishes eTreg lineage and neonatal immune tolerance by targeting Hadha-mediated fatty acid oxidation.
HADHA alleviates hepatic steatosis and oxidative stress in NAFLD via inactivation of the MKK3/MAPK pathway.
Title: HADHA alleviates hepatic steatosis and oxidative stress in NAFLD via inactivation of the MKK3/MAPK pathway.
UBE2O promotes lipid metabolic reprogramming and liver cancer progression by mediating HADHA ubiquitination.
Title: UBE2O promotes lipid metabolic reprogramming and liver cancer progression by mediating HADHA ubiquitination.
The mitochondrial beta-oxidation enzyme HADHA restrains hepatic glucagon response by promoting beta-hydroxybutyrate production.
Title: The mitochondrial β-oxidation enzyme HADHA restrains hepatic glucagon response by promoting β-hydroxybutyrate production.
The enzyme activity of mitochondrial trifunctional protein is not altered by lysine acetylation or lysine succinylation.
Title: The enzyme activity of mitochondrial trifunctional protein is not altered by lysine acetylation or lysine succinylation.
Cardioprotective GLP-1 metabolite prevents ischemic cardiac injury by inhibiting mitochondrial trifunctional protein-alpha.
Title: Cardioprotective GLP-1 metabolite prevents ischemic cardiac injury by inhibiting mitochondrial trifunctional protein-α.
These findings suggested that palmitic acid-induced autophagy supports the survival of IECs. Taken together, these results suggested that HADHA is involved in long-chain fatty acid-induced autophagy in IECs, thus providing new insights into the pathology of IBD and revealing novel therapeutic targets of IBD.
Title: HADHA, the alpha subunit of the mitochondrial trifunctional protein, is involved in long-chain fatty acid-induced autophagy in intestinal epithelial cells.
nonstructural protein 5 (NS5) interacted with hydroxyacyl-CoA dehydrogenase alpha and beta subunits, two components of the mitochondrial trifunctional protein (MTP) involved in LCFA beta-oxidation
Title: Japanese encephalitis virus nonstructural protein NS5 interacts with mitochondrial trifunctional protein and impairs fatty acid β-oxidation.
alphaTFP may modulate CL molecular species composition in murine heart and liver. In addition, L4-CL might not be an essential requirement for mitochondrial supercomplex assembly.
Title: Differential reduction in cardiac and liver monolysocardiolipin acyltransferase-1 and reduction in cardiac and liver tetralinoleoyl-cardiolipin in the α-subunit of trifunctional protein heterozygous knockout mice.
Heterozygosity for beta-oxidation defects predisposes to fatty live disease and insulin resistance in aging mice
Title: Mice heterozygous for a defect in mitochondrial trifunctional protein develop hepatic steatosis and insulin resistance.

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Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics (MGI)

Endonuclease-mediated (2)
Targeted (1) 1 citation

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

Hadha (e-PCR), detects polymorphism
- UniSTS:506965

RH143343 (e-PCR)
- UniSTS:232590

D12S1229E (e-PCR)
- UniSTS:151476

RH126081 (e-PCR)
- UniSTS:162793

Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables 3-hydroxyacyl-CoA dehydratase activity	IEA Inferred from Electronic Annotation more info
enables 3-hydroxyacyl-CoA dehydratase activity	ISO Inferred from Sequence Orthology more info
enables 3-hydroxyacyl-CoA dehydrogenase activity	ISO Inferred from Sequence Orthology more info
enables NAD binding	ISO Inferred from Sequence Orthology more info
enables NAD+ binding	IEA Inferred from Electronic Annotation more info
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 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 enoyl-CoA hydratase activity	TAS Traceable Author Statement more info	PubMed
enables fatty-acyl-CoA binding	IEA Inferred from Electronic Annotation more info
enables fatty-acyl-CoA binding	ISO Inferred from Sequence Orthology more info
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 protein binding	IPI Inferred from Physical Interaction more info	PubMed
enables protein-containing complex binding	IEA Inferred from Electronic Annotation more info
enables protein-containing complex binding	ISO Inferred from Sequence Orthology more info

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	IEA Inferred from Electronic Annotation more info
acts_upstream_of_or_within fatty acid beta-oxidation	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in fatty acid beta-oxidation	ISO Inferred from Sequence Orthology more info
acts_upstream_of_or_within response to insulin	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in response to xenobiotic stimulus	IEA Inferred from Electronic Annotation more info
involved_in response to xenobiotic stimulus	ISO Inferred from Sequence Orthology more info

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	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	HDA more info	PubMed
located_in mitochondrial inner membrane	ISO Inferred from Sequence Orthology 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 mitochondrion	HDA more info	PubMed
located_in mitochondrion	ISO Inferred from Sequence Orthology more info
located_in mitochondrion	TAS Traceable Author Statement more info	PubMed

General protein information

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Preferred Names: trifunctional enzyme subunit alpha, mitochondrial

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

NP_849209.1

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_178878.3 → NP_849209.1 trifunctional enzyme subunit alpha, mitochondrial precursor

See identical proteins and their annotated locations for NP_849209.1

Status: VALIDATED

Source sequence(s)

AK029017

Consensus CDS

CCDS19155.1

UniProtKB/Swiss-Prot

Q3TCY3, Q5U5Y5, Q8BMS1, Q8QZU4

Related

ENSMUSP00000120976.2, ENSMUST00000156859.3

Conserved Domains (1) summary

TIGR02441
Location:27 → 762

fa_ox_alpha_mit; fatty acid oxidation complex, alpha subunit, mitochondrial