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20-September-2008 09:55:58 - Mitochondrial trifunctional protein Schematic demonstrating mitochondrial fatty acid beta-oxidation and effects of LCHAD deficiency Schematic demonstrating mitochondrial fatty acid beta-oxidation and effects of LCHAD deficiency Mitochondrial trifunctional protein is a protein which catalyzes several reactions in beta oxidation. It has two subunits: HADHA HADHB Pathology Disorders are associated with: Mitochondrial trifunctional protein deficiency LCHAD deficiency 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 This protein-related article is a stub. Retrieved from http://en..org/wiki/Mitochondrial_trifunctional_protein Categories: Proteins | Protein stubs Views Article Discussion this page History Personal tools Log in / create account Navigation Main page Contents Featured content Current events Random article Search Go Search Interaction Community portal Recent changes Contact Donate to Help Toolbox What links here Related changes Upload file Special pages Printable version Permanent link Cite this page This page was last modified on 29 April 2007, at 20:3
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