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14-September-2008 10:43:23 - Mitochondrial trifunctional protein deficiency January 2008 Mitochondrial trifunctional protein deficiency Classification and external resources ICD-9 272.8 OMIM 609015 DiseasesDB 34111 eMedicine ped/1284 Mitochondrial trifunctional protein deficiency is an autosomal recessive fatty acid oxidation disorder1 that prevents the body from converting certain fats to energy, particularly during periods without food . People with this disorder have inadequate levels of an enzyme that breaks down a certain group of fats called long-chain fatty acids. Contents 1 Diagnosis 2 Genetics 3 See also 4 External links 5 References Diagnosis Onset of this disorder may begin during infancy or later in life. Signs and symptoms of the early onset form can include feeding difficulties, lack of energy lethargy, low blood sugar hypoglycemia, muscle weakness hypotonia, liver problems, and a high risk for complications such as life-threatening heart and breathing problems, coma, and sudden unexpected death. The late-onset form is usually less severe; signs and symptoms can include hypotonia, muscle pain, a breakdown of muscle tissue, and abnormalities in the nervous system that affect arms and legs peripheral neuropathy. Episodes of mitochondrial trifunctional protein deficiency can be triggered by periods of fasting or by illnesses such as viral infections. Diagnosis of this disorder is often confirmed using tandem mass spectrometry.1 Genetics Mitochondrial trifunctional protein deficiency has an autosomal recessive pattern of inheritance. Mitochondrial trifunctional protein deficiency has an autosomal recessive pattern of inheritance. Mutations in the HADHA or HADHB genes can lead to inadequate levels of an enzyme complex known as mitochondrial trifunctional protein. Long-chain fatty acids from food and body fat cannot be metabolized and processed without sufficient levels of this enzyme complex. As a result, these fatty acids are not converted to energy, which can lead to characteristic features of this disorder, such as lethargy and hypoglycemia. Long-chain fatty acids or partially metabolized fatty acids may build up in tissues and damage the liver, heart, and muscles, causing more serious complications. See also This article incorporates public domain text from The U.S. National Library of Medicine External links Overview of condition at NLM Genetics Home Reference References ^ a b Solish JO, Singh RH 2002. Management of fatty acid oxidation disorders: a survey of current treatment strategies. J Am Diet Assoc. 102 12: 1800-1803. doi:10.1016/S0002-82230290386-X. PMID 12487544. 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 This genetic disorder article is a stub. Retrieved from http://en..org/wiki/Mitochondrial_trifunctional_protein_deficiency Categories: Metabolic disorders | Genetic disorders | Autosomal recessive disorders | Inborn errors of metabolism | Genetic disorder stubsHidden category: Articles needing additional references from January 2008 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 27 May 2008, at 19
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