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

Structure: - consists of: - trifunctional enzyme alpha subunit (HADHA) - trifunctional enzyme beta subunit (HADHB) Pathology: - deficiency of all 3 enzyme activities associated with trifunctional protein deficiency

General

mitochondrial protein molecular complex

Properties

COMPARTMENT: mitochondria SUBUNITS: long chain enoyl CoA hydratase/dehydrogenase COMPARTMENT: mitochondrial matrix MOTIF: glutamate residue {E151} glutamate residue {E173} Ser phosphorylation site {S756} trifunctional enzyme beta subunit, mitochondrial MOTIF: cysteine residue {C138} histidine residue {H428} cysteine residue {C458}

References

UniProt :accession Q15388

Components

trifunctional enzyme beta subunit, mitochondrial; TP-beta; hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase beta subunit (HADHB) 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)