Mitochondrial trifunctional protein

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Mitochondrial trifunctional protein is a protein which catalyzes several reactions in beta oxidation. It has two subunits:

• HADHA
• HADHB

[edit] Pathology

Disorders are associated with:

• Mitochondrial trifunctional protein deficiency
• LCHAD deficiency

[edit] External links

• MeSH mitochondrial+trifunctional+protein+TP

v • d • e Enzymes: multienzyme complexes CAD (Carbamoyl phosphate synthase II, Aspartate carbamoyltransferase, Dihydroorotase) - Cholesterol side-chain cleavage enzyme - Cytochrome b6f complex - Electron transport chain - Fatty acid synthetase complex - Glycine decarboxylase complex - Mitochondrial trifunctional protein (HADHA, HADHB) - Oxoglutarate dehydrogenase - Phosphoenolpyruvate sugar phosphotransferase system - Photosynthetic reaction center complex proteins - Photosystem - Polyketide synthase - Pyruvate dehydrogenase complex (E1, E2, E3) - Sucrase-isomaltase complex -Tryptophan synthase

v • d • e Metabolism: lipid metabolism - triglyceride and fatty acid enzymes Lipid production ATP citrate lyase - Acetyl-CoA carboxylase Fatty acid synthesis/Fatty acid synthase: Beta-ketoacyl-ACP synthase - Β-Ketoacyl ACP reductase - 3-Hydroxyacyl ACP dehydrase - Enoyl ACP reductase triacyl glycerol: Glycerol-3-phosphate dehydrogenase - Thiokinase Lipid degradation Acyl transport: Carnitine palmitoyltransferase I - Carnitine-acylcarnitine translocase - Carnitine palmitoyltransferase II Beta oxidation/Mitochondrial trifunctional protein: Acyl CoA dehydrogenase (ACADL, ACADM, ACADS, ACADVL) - Enoyl-CoA hydratase - 3-Hydroxyacyl CoA dehydrogenase - Acetyl-CoA C-acyltransferase to acetyl-CoA: Malonyl-CoA decarboxylase unsaturated (Enoyl CoA isomerase, 2,4 Dienoyl-CoA reductase) odd chain (Propionyl-CoA carboxylase) aldehydes (Long-chain-aldehyde dehydrogenase) see also disorders