Bos taurus Gene: HADHA
Summary
InnateDB Gene IDBG-637102.3
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol HADHA
Gene Name trifunctional enzyme subunit alpha, mitochondrial precursor
Synonyms
Species Bos taurus
Ensembl Gene ENSBTAG00000015038
Encoded Proteins
trifunctional enzyme subunit alpha, mitochondrial
Protein Structure
Useful resources Stemformatics EHFPI ImmGen
Entrez Gene
Summary This gene does not have any Entrez summary - the following is the summary from its human ortholog ENSG00000084754:
This gene encodes the alpha subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the alpha subunit catalyzing the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities. Mutations in this gene result in trifunctional protein deficiency or LCHAD deficiency. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation. [provided by RefSeq, Jul 2008]
Gene Information
Type Protein coding
Genomic Location Chromosome 11:73246485-73288803
Strand Forward strand
Band
Transcripts
ENSBTAT00000020020 ENSBTAP00000020020
Interactions
Number of Interactions This gene and/or its encoded proteins are associated with 0 experimentally validated interaction(s) in this database.
They are also associated with 51 interaction(s) predicted by orthology.
Predicted by orthology
Total 51 [view]
Gene Ontology

Molecular Function
Accession GO Term
GO:0003824 catalytic activity
GO:0003857 3-hydroxyacyl-CoA dehydrogenase activity
GO:0004300 enoyl-CoA hydratase activity
GO:0005515 protein binding
GO:0016491 oxidoreductase activity
GO:0016509 long-chain-3-hydroxyacyl-CoA dehydrogenase activity
GO:0016616 oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor
GO:0050662 coenzyme binding
Biological Process
GO:0006631 fatty acid metabolic process
GO:0006635 fatty acid beta-oxidation
GO:0008152 metabolic process
GO:0032868 response to insulin
GO:0055114 oxidation-reduction process
Cellular Component
GO:0005739 mitochondrion
GO:0005743 mitochondrial inner membrane
GO:0016507 mitochondrial fatty acid beta-oxidation multienzyme complex
GO:0042645 mitochondrial nucleoid
Orthologs
Species
Homo sapiens
Mus musculus
Gene ID
Gene Order
Pathway Predictions based on Human Orthology Data
NETPATH
REACTOME
Acyl chain remodeling of CL pathway
Beta oxidation of hexanoyl-CoA to butanoyl-CoA pathway
Beta oxidation of myristoyl-CoA to lauroyl-CoA pathway
Beta oxidation of palmitoyl-CoA to myristoyl-CoA pathway
Beta oxidation of lauroyl-CoA to decanoyl-CoA-CoA pathway
Beta oxidation of octanoyl-CoA to hexanoyl-CoA pathway
Beta oxidation of decanoyl-CoA to octanoyl-CoA-CoA pathway
Fatty acid, triacylglycerol, and ketone body metabolism pathway
Metabolism of lipids and lipoproteins pathway
Mitochondrial Fatty Acid Beta-Oxidation pathway
Glycerophospholipid biosynthesis pathway
Phospholipid metabolism pathway
mitochondrial fatty acid beta-oxidation of saturated fatty acids pathway
Metabolism pathway
Acyl chain remodeling of CL pathway
Fatty acid, triacylglycerol, and ketone body metabolism pathway
Mitochondrial Fatty Acid Beta-Oxidation pathway
Beta oxidation of lauroyl-CoA to decanoyl-CoA-CoA pathway
Metabolism pathway
Beta oxidation of octanoyl-CoA to hexanoyl-CoA pathway
Metabolism of lipids and lipoproteins pathway
Beta oxidation of decanoyl-CoA to octanoyl-CoA-CoA pathway
mitochondrial fatty acid beta-oxidation of saturated fatty acids pathway
Beta oxidation of hexanoyl-CoA to butanoyl-CoA pathway
Beta oxidation of myristoyl-CoA to lauroyl-CoA pathway
Glycerophospholipid biosynthesis pathway
Phospholipid metabolism pathway
Beta oxidation of palmitoyl-CoA to myristoyl-CoA pathway
KEGG
Valine, leucine and isoleucine degradation pathway
Butanoate metabolism pathway
Propanoate metabolism pathway
Biosynthesis of unsaturated fatty acids pathway
Tryptophan metabolism pathway
Fatty acid degradation pathway
Fatty acid elongation pathway
Lysine degradation pathway
beta-Alanine metabolism pathway
beta-Alanine metabolism pathway
Biosynthesis of unsaturated fatty acids pathway
Valine, leucine and isoleucine degradation pathway
Fatty acid elongation pathway
Lysine degradation pathway
Tryptophan metabolism pathway
Propanoate metabolism pathway
Butanoate metabolism pathway
Fatty acid degradation pathway
INOH
Tryptophan degradation pathway
Propanoate metabolism pathway
Butanoate metabolism pathway
Valine Leucine Isoleucine degradation pathway
Lysine degradation pathway
PID NCI
Cross-References
SwissProt
TrEMBL Q3SZ00
UniProt Splice Variant
Entrez Gene 281810
UniGene Bt.48598
RefSeq NM_174335
HUGO
OMIM
CCDS
HPRD
IMGT
EMBL BC103307 DAAA02031607 DAAA02031608
GenPept AAI03308
RNA Seq Atlas 281810