trifunctional enzyme subunit alpha, mitochondrial; TP-alpha; 78 kD gastrin-binding protein [includes: long-chain enoyl-CoA hydratase; long chain 3-hydroxyacyl-CoA dehydrogenase] (HADHA, HADH)

Function

• bifunctional subunit of the trifunctional enzyme complex
• octamer of 4 alpha (HADHA) & 4 beta (HADHB) subunits
• fatty acid beta-oxidation cycle; step 2
• fatty acid beta-oxidation cycle; step 3

(3S)-3-hydroxyacyl-CoA <--> trans-2(or 3)-enoyl-CoA + H2O

(S)-3-hydroxyacyl-CoA + NAD+ <--> 3-oxoacyl-CoA + NADH

Structure

• in the N-terminal section; belongs to the enoyl-CoA hydratase/isomerase family CoA dehydrogenase family
• in the central section; belongs to the 3-hydroxyacyl- CoA dehydrogenase family

Compartment

mitochondria

Pathology

• defects in HADHA are a cause of trifunctional protein deficiency
• defects in HADHA are the cause of long-chain 3-hydroxyl-CoA dehydrogenase deficiency
• defects in HADHA are a cause of maternal acute fatty liver of pregnancy

Laboratory

• HADHA gene mutation

More general terms

• enol CoA hydratase
• 3-OH acyl CoA dehydrogenase

Component of

• mitochondrial trifunctional enzyme complex (trifunctional protein complex, TFP complex, trifunctional enzyme)

Database

• UniProt: http://www.uniprot.org/uniprot/P40939.html
• OMIM: https://mirror.omim.org/entry/600890