Features Interactions Isoform Disease Linear motifs Fingerprint Network All partners

ECHA_HUMAN

Mitochondrial trifunctional enzyme catalyzes the last three of the four reactions of the mitochondrial beta-oxidation pathway (PubMed, PubMed, PubMed, PubMed). 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 (PubMed). Among the enzymes involved in this pathway, the trifunctional enzyme exhibits specificity for long-chain fatty acids (PubMed). 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 (PubMed, PubMed, PubMed). Independently of the subunit beta, the trifunctional enzyme subunit alpha/HADHA also has a monolysocardiolipin acyltransferase activity (PubMed). 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 (PubMed). Allows the acylation of monolysocardiolipin with different acyl-CoA substrates including oleoyl-CoA for which it displays the highest activity (PubMed). [View more on UniProt]

0100200300400500600700
Transmembrane
Phase separation
ELM
Phosphorylation
PFAM
Coiled coil
Anchor
Disordered
Interacting regions
Sequence
LOADING 91%

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0100200300400500600700
ECHA_HUMAN
LOADING 67%
0100200300400500600700
ECHA_HUMAN
LOADING 67%
Download full PS network for entry.
0100200300400500600700800
Interacting regions
Canonical [ECHA_HUMAN]
Isoform [A0A2R8Y688] alignment
Isoform [A0A2R8Y4E0] alignment
Isoform [A0A2R8Y4F5] alignment
Isoform [A0A2R8YDM1] alignment
Isoform [A0A2R8YG21] alignment
Isoform [H0YFD6] alignment
Isoform [P40939-2] alignment
LOADING 53%

No data found.

No annotated instance was found. To search for linear motifs, use the ELM prediction server.

Molecular function

Biological process

Disease

No data found.

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