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Numerous genetic disorders are caused by errors in fatty acid metabolism. These disorders may be described as fatty oxidation disorders or as a lipid storage disorders, and are any one of several inborn errors of metabolism that result from enzyme defects affecting the ability of the body to oxidize fatty acids in order to produce energy within muscles, liver, and other cell types. The following codes are used with International Statistical Classification of Diseases and Related Health Problems. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... Wikipedia does not have an article with this exact name. ... Inborn errors of metabolism comprise a large class of genetic diseases involving disorders of metabolism. ... A genetic disorder, or genetic disease is a disease caused, at least in part, by the genes of the person with the disease. ... Lipid storage disorders (or lipidoses) are a group of inherited metabolic disorders in which harmful amounts lipids (fats) accumulate in some of the body’s cells and tissues. ... Inborn errors of metabolism are a large group of rare but often serious metabolic disorders that generally arise from deficiency or malfunction of enzymes that regulate conversion of various substances into others. ... To oxidize an element or a compound is to increase its oxidation number. ... In chemistry, especially biochemistry, a fatty acid is a carboxylic acid (or organic acid), often with a long aliphatic tail (long chains), either saturated or unsaturated. ... Cells in culture, stained for keratin (red) and DNA (green) The cell is the structural and functional unit of all living organisms, and are sometimes called the building blocks of life. ...

Some of the more common fatty acid metabolism disorders are:

Coenzyme A dehydrogenase deficiencies

• Very long-chain acyl-coenzyme A dehydrogenase deficiency (VLCAD)
• Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency (LCHAD)
• Medium-chain acyl-coenzyme A dehydrogenase deficiency (MCAD)
• Short-chain acyl-coenzyme A dehydrogenase deficiency (SCAD)
• Short chain L-3-hydroxyacyl-coA dehydrogenase deficiency (SCHAD)

Acetyl-CoA (CoA with acetate highlighted) Coenzyme A (CoA, CoASH, or HSCoA) is adapted from β-mercaptoethylamine, panthothenate and adenosine triphosphate and used in metabolism in areas such as fatty acid oxidization and the citric acid cycle. ... A dehydrogenase is an enzyme that oxidizes a substrate by transferring one or more protons and a pair of electrons to an acceptor, usually NAD/NADP or a flavin coenzyme such as FAD or FMN. Common examples of dehydrogenase enzymes in the TCA cycle are pyruvate dehydrogenase, isocitrate dehydrogenase, and... Very long-chain acyl-coenzyme A dehydrogenase deficiency is a condition that prevents the body from converting certain fats to energy, particularly during periods without food. ... Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency, often shortened to LCHAD deficiency is a rare genetic disorder that prevents the body from converting certain fats to energy, particularly during periods of fasting. ... Medium-chain acyl-coenzyme A dehydrogenase deficiency is one of a group of conditions that is associated with inborn errors of metabolism in fatty acid oxidation. ...

Other Coenzyme A enzyme deficiencies

• 2,4 Dienoyl-coA reductase deficiency
• 3-hydroxy-3-methylglutaryl-CoA lyase deficiency

Acetyl-CoA (CoA with acetate highlighted) Coenzyme A (CoA, CoASH, or HSCoA) is adapted from β-mercaptoethylamine, panthothenate and adenosine triphosphate and used in metabolism in areas such as fatty acid oxidization and the citric acid cycle. ... 3-hydroxy-3-methylglutaryl-CoA lyase deficiency also referred to as HMG-CoA lyase deficiency, is an uncommon inherited disorder in which the body cannot properly process the amino acid leucine. ...

Carnitine related

• Primary carnitine deficiency
• Carnitine-acylcarnitine translocase deficiency
• Carnitine palmitoyltransferase I deficiency (CPT)
• Carnitine palmitoyltransferase II deficiency (CPT)

This article contains information that has not been verified and thus might not be reliable. ... Primary carnitine deficiency is a condition that prevents the body from using fats for energy, particularly during periods without food. ... Carnitine-acylcarnitine translocase deficiency is a rare condition that prevents the body from converting long-chain fatty acids into energy, particularly during periods without food. ... Carnitine palmitoyltransferase I deficiency is a rare condition that prevents the body from converting certain fats called long-chain fatty acids into energy, particularly during periods without food. ... Carnitine palmitoyltransferase II deficiency is a condition that prevents the body from converting certain fats called long-chain fatty acids into energy, particularly during periods without food. ...

other

• Mitochondrial trifunctional protein deficiency
• Electron transfer flavoprotein (ETF) dehydrogenase deficiency (GAII & MADD)