Carnitine-acylcarnitine translocase: Difference between revisions
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'''Carnitine-acylcarnitine translocases''' are responsible for transporting both [[cartinine]] and [[carnitine]]-[[fattyacid]] complexes into and out of the [[mitochondria]], across the inner mitochondrial membrane. 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 [[cartinine]] by the use of the enzyme [[cartinine 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. |
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'''Carnitine-acylcarnitine translocase''' is responsible for transporting [[carnitine]] into the [[mitochondria]]. A disorder is associated with [[carnitine-acylcarnitine translocase deficiency]]. |
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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) |
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[[Image:Acyl-CoA from cytosol to the mitochondrial matrix.gif|thumb|center|400px|Acyl-CoA from cytosol to the mitochondrial matrix.gif]] |
[[Image:Acyl-CoA from cytosol to the mitochondrial matrix.gif|thumb|center|400px|Acyl-CoA from cytosol to the mitochondrial matrix.gif]] |
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Revision as of 15:45, 25 November 2007
| solute carrier family 25 (carnitine/acylcarnitine translocase), member 20 | |||||||
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| Identifiers | |||||||
| Symbol | SLC25A20 | ||||||
| Alt. symbols | CACT | ||||||
| NCBI gene | 788 | ||||||
| HGNC | 1421 | ||||||
| OMIM | 212138 | ||||||
| RefSeq | NM_000387 | ||||||
| UniProt | O43772 | ||||||
| Other data | |||||||
| Locus | Chr. 3 p21.31 | ||||||
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Carnitine-acylcarnitine translocases are responsible for transporting both cartinine and carnitine-fattyacid complexes into and out of the mitochondria, across the inner mitochondrial membrane. 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 cartinine by the use of the enzyme cartinine 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.
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)
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