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20-September-2008 09:55:58 - Carnitine-acylcarnitine translocase solute carrier family 25 carnitine/acylcarnitine translocase, member 20 Identifiers Symbol SLC25A20 Alt. Symbols CACT Entrez 788 HUGO 1421 OMIM 212138 RefSeq NM_000387 UniProt O43772 Other data Locus Chr. 3 p21.31 Carnitine-acylcarnitine translocases are responsible for transporting both carnitine and carnitine-fatty acid complexes into and out of the mitochondria, across the inner mitochondrial membrane. Function This enzyme is required as fatty acids cannot cross the mitchondrial membranes without assistance. The fatty acid is firstly bound to CoA to cross the external mitochondrial membrane. It then switches the CoA for carnitine by the use of the enzyme carnitine palmitoyl transferase I. The complex then uses facilitated diffusion by Carnitine-acylcarnitine translocase to enter the mitochondrial matrix. Here, the acylcartinine complex is disrupted by cartinine palmitoyl transferase II and the fatty acid rebinds to CoA. Cartinine then diffuses back across the membrane by a second carnitine-acylcarnitine translocase into the inter-mitochondiral membrane space. This is the cartinine shuttle system. Clinical significance A disorder is associated with carnitine-acylcarnitine translocase deficiency. This disorder prevents the shuttle-like action of cartinine from assisting fatty acids across the mitochondiral membrane and therefore there is decreased fatty acid metabolism. The result of this is an increased number of fat droplets within muscles and liver, decreased tolerance to long term excerise, inability to fast for more than a few hours, muscle weakness and wasting, strong acidic smell on breath due to protein breakdown Acyl-CoA from cytosol to the mitochondrial matrix.gif Acyl-CoA from cytosol to the mitochondrial matrix.gif This membrane protein-related article is a stub. v d e Membrane proteins, carrier proteins: membrane transport proteins ABC-transporter A1, A2, A3, A4, A7, A8, A12, B1 B2-3, B2, B3, B4, B5, B6, B7, B9, B11 C1, C2, C3, C4, C5, C6, C8, C8-9, C10, C13, C11 D1, D2, D3, D4, E1, F1, F2 G1, G2, G4, Sterolin G5, G8 Solute carrier 1A1-7, 1A1, 1A2, 1A3, 1A4, 1A5, 2A1, 2A2, 2A3, 2A4, 2A5, 2A6, 2A8, 2A9, 2A10, 2A12, 3A1, 3A2, 4A1, 4A2, 4A3, 4A4, 4A5, 4A7, 4A8, 4A11, 5A1-2, 5A1, 5A3, 5A5, 5A8, 6A1, 6A2, 6A3, 6A4, 6A5, 6A8, 6A9, 6A19, 7A1, 7A2, 7A3, 7A4, 7A5, 7A7, 7A8, 7A9, 7A11, 8A1-3, 9A1, 9A2, 9A3, 9A3R1, 9A3R2, 9A5, 9A6, 9A8, 10A1, 10A2, 10A3, 10A7, 11A1, 11A2, 11A3, 12A1-2, 12A3, 12A4, 12A5, 12A6, 12A7, 14A1, 13A3, 14A2, 15A1, 15A2, 16A1, 16A2, 16A3, 16A4, 17A1, 17A5, 17A6-8, 17A7, 18A1, 18A2, 18A3, 19A1, 19A2, 19A3, 20A1, 20A2, 22A1, 22A2, 22A3, 22A4, 22A5, 22A6, 22A7, 22A9, 22A11, 22A12, 22A18, 23A1, 23A2, 24A1-2, 24A5, 25A1, 25A3, 25A4, 25A4-6, 25A8, 25A10, 25A11, 25A12, 25A13, 25A14, 25A15, 25A17, 25A19, 25A20, 25A27, 25A31, 25A37, 25A39, 26A2, 26A3, 26A4, 26A5, 26A6, 26A7, 26A8, 27A1, 27A2, 27A3, 27A4, 28A1, 28A2, 29A1, 29A2, 29A4, 30A4, 30A7, 30A8, 31A1, 31A2, 32A1, 34A1, 34A2, 34A3, 35A1, 35A2, 35B2, 35B4, 35C1, 36A1, 37A4, 38A2, 38A3, 39A1, 39A2, 39A3, 39A4, 39A6, 39A7, 40A1, 43A1 44A1, 44A2, 44A4, 45A2, O1A2, O1B1, O1B3, O2B1, O431, O4A1 Monosaccharide GLUT1, GLUT2, GLUT3, GLUT4, GLUT5, GLUT6, GLUT8, GLUT9 Other Amino acid CD98 - Fatty acid CD36 - Ion channels - Ion pumps - Mitochondrial membrane transport protein - Neurotransmitter transport proteins - Nuclear Importin, Karyopherin 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 v d e Mitochondrial enzymes and transporters Outer membrane fatty acid degradation Carnitine palmitoyltransferase I, Long fatty acyl CoA synthetase tryptophan metabolism Kynureninase monoamine neurotransmitter metabolism Monoamine oxidase Intermembrane space Adenylate kinase - Creatine kinase Inner membrane oxidative phosphorylation Coenzyme Q - cytochrome c reductase, Cytochrome c, NADH dehydrogenase, Succinate dehydrogenase pyrimidine metabolism Dihydroorotate dehydrogenase mitochondrial shuttle Malate-aspartate shuttle, Glycerol phosphate shuttle other Glutamate aspartate transporter, Glycerol-3-phosphate dehydrogenase, ATP synthase, Carnitine palmitoyltransferase II Matrix citric acid cycle Citrate synthase, Aconitase, Isocitrate dehydrogenase, Oxoglutarate dehydrogenase, Succinyl coenzyme A synthetase, Fumarase, Malate dehydrogenase anaplerotic reactions Aspartate transaminase, Glutamate dehydrogenase, Pyruvate dehydrogenase complex urea cycle Carbamoyl phosphate synthetase I, Ornithine transcarbamylase, N-Acetylglutamate synthase alcohol metabolism ALDH2 Other/to be sorted Cholesterol side-chain cleavage enzyme Mitochondrial DNA Complex I 7 units - Complex III 1 unit - Complex IV 3 units - ATP synthase 2 units Retrieved from http://en..org/wiki/Carnitine-acylcarnitine_translocase Categories: Genes on chromosome 3 | Membrane protein stubs | Solute carrier familyHidden category: Protein pages needing a picture 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 31 May 2008, at 12:58
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