HADHA report

I. Expression across cell types

II. Expression across tissues

III. Associated gene sets

GO_0032868Biological processresponse to insulin
GO_0006635Biological processfatty acid beta-oxidation
GO_0009410Biological processresponse to xenobiotic stimulus
GO_0035965Biological processcardiolipin acyl-chain remodeling
GO_0016507Cellular componentmitochondrial fatty acid beta-oxidation multienzyme complex
GO_0042645Cellular componentmitochondrial nucleoid
GO_0005743Cellular componentmitochondrial inner membrane
GO_0005739Cellular componentmitochondrion
GO_0044877Molecular functionprotein-containing complex binding
GO_0003857Molecular function3-hydroxyacyl-CoA dehydrogenase activity
GO_0003985Molecular functionacetyl-CoA C-acetyltransferase activity
GO_0070403Molecular functionNAD+ binding
GO_0004300Molecular functionenoyl-CoA hydratase activity
GO_0000062Molecular functionfatty-acyl-CoA binding
GO_0016509Molecular functionlong-chain-3-hydroxyacyl-CoA dehydrogenase activity
GO_0005515Molecular functionprotein binding
GO_0018812Molecular function3-hydroxyacyl-CoA dehydratase activity

IV. Literature review

[source]
Gene nameHADHA
Protein nameHydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha
Trifunctional enzyme subunit alpha, mitochondrial (78 kDa gastrin-binding protein) (Monolysocardiolipin acyltransferase) (EC 2.3.1.-) (TP-alpha) [Includes: Long-chain enoyl-CoA hydratase (EC 4.2.1.17); Long chain 3-hydroxyacyl-CoA dehydrogenase (EC 1.1.1.211)]
enoyl-CoA hydratase (EC 4.2.1.17)
SynonymsHADH
DescriptionFUNCTION: Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway . The mitochondrial beta-oxidation pathway is the major energy-producing process in tissues and is performed through four consecutive reactions breaking down fatty acids into acetyl-CoA . Among the enzymes involved in this pathway, the trifunctional enzyme exhibits specificity for long-chain fatty acids . Mitochondrial trifunctional enzyme is a heterotetrameric complex composed of two proteins, the trifunctional enzyme subunit alpha/HADHA described here carries the 2,3-enoyl-CoA hydratase and the 3-hydroxyacyl-CoA dehydrogenase activities while the trifunctional enzyme subunit beta/HADHB bears the 3-ketoacyl-CoA thiolase activity . Independently of the subunit beta, the trifunctional enzyme subunit alpha/HADHA also has a monolysocardiolipin acyltransferase activity . It acylates monolysocardiolipin into cardiolipin, a major mitochondrial membrane phospholipid which plays a key role in apoptosis and supports mitochondrial respiratory chain complexes in the generation of ATP . Allows the acylation of monolysocardiolipin with different acyl-CoA substrates including oleoyl-CoA for which it displays the highest activity . .

AccessionsA0A2R8Y688
P40939
ENST00000643233.1
ENST00000643057.1
ENST00000645274.1
A0A2R8Y4E0
A0A2R8Y4F5
ENST00000492433.2
ENST00000643063.1
A0A2R8YDM1
ENST00000644428.1
H0YFD6
ENST00000646031.1
A0A2R8YG21
ENST00000646483.1 [P40939-2]
ENST00000380649.8 [P40939-1]