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14-September-2008 10:43:22 - I deficiency February 2008 Carnitine palmitoyltransferase I deficiency Classification and external resources Carnitine ICD-9 277.85 OMIM 255120 DiseasesDB 32535 eMedicine ped/321 Carnitine palmitoyltransferase I deficiency is a rare metabolic disorder that prevents the body from converting certain fats called long-chain fatty acids into energy, particularly during periods without food. Carnitine, a natural substance acquired mostly through the diet, is used by cells to process fats and produce energy. People with this disorder have a faulty enzyme that prevents these long-chain fatty acids from being transported into the mitochondria to be broken down. Contents 1 Symptoms 2 Differential diagnosis 3 Genetics 4 See also 5 External links Symptoms Signs and symptoms of this disorder include low levels of ketones products of fat breakdown that are used for energy and low blood sugar hypoglycemia. Together these signs are called hypoketotic hypoglycemia. People with this disorder typically also have an enlarged liver hepatomegaly, muscle weakness, and elevated levels of carnitine in the blood. Differential diagnosis This condition is sometimes mistaken for Reye syndrome, a severe disorder that develops in children while they appear to be recovering from viral infections such as chicken pox or flu. Most cases of Reye syndrome are associated with the use of aspirin during these viral infections. Genetics Carnitine palmitoyltransferase I deficiency has an autosomal recessive pattern of inheritance. Carnitine palmitoyltransferase I deficiency has an autosomal recessive pattern of inheritance. Mutations in the CPT1A gene cause carnitine palmitoyltransferase I deficiency. Mutations in the CPT1A gene lead to the production of a defective version of an enzyme called carnitine palmitoyltransferase I. Without this enzyme, long-chain fatty acids from food and fats stored in the body cannot be transported into mitochondria to be broken down and processed. As a result, excessive levels of long-chain fatty acids may build up in tissues, damaging the liver, heart, and brain. This condition has an autosomal recessive inheritance pattern, which means the defective gene is located on an autosome, and two copies of the gene - one from each parent - must be inherited to be affected by the disorder. The parents of a child with an autosomal recessive disorder are carriers of one copy of the defective gene, but are usually not affected by the disorder. See also Primary carnitine deficiency Carnitine palmitoyltransferase II deficiency External links This article incorporates public domain text from The U.S. National Library of Medicine v d e Lipid metabolism disorders / Inborn error of lipid metabolism - dyslipidemia E78 and E71.3, 272 Hyperlipidemia Hypercholesterolemia/Hypertriglyceridemia Familial hypercholesterolemia, Combined hyperlipidemia - Xanthoma Hypolipoproteinemia Hypoalphalipoproteinemia/HDL Lecithin cholesterol acyltransferase deficiency, Tangier disease Hypobetalipoproteinemia/LDL Abetalipoproteinemia, Apolipoprotein B deficiency Lipodystrophy Barraquer-Simons syndrome Fatty acid metabolism deficiency transport: Carnitine Primary, I, II, -acylcarnitine - Adrenoleukodystrophy beta oxidation: Acyl CoA dehydrogenase Short-chain, Medium-chain, Long-chain 3-hydroxy, Very long-chain - Mitochondrial trifunctional protein deficiency to acetyl-CoA: Malonic aciduria Cholesterol synthesis Smith-Lemli-Opitz syndrome Other Sjögren-Larsson syndrome - Lipomatosis - Adiposis dolorosa - Lipoid proteinosis see also lipid metabolism enzymes, lipoprotein metabolism Retrieved from http://en..org/wiki/Carnitine_palmitoyltransferase_I_deficiency Categories: Metabolic disorders | Genetic disorders | Autosomal recessive disorders | Rare diseases | Hepatology | Inborn errors of metabolismHidden categories: Articles lacking sources from February 2008 | All articles lacking sources 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 23 April 2008, at 12:59
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