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[DOC File]Nutricia North America
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Enfaport is a medical food designed for children with defects in the hydrolysis of long-chain fat. 83% of the fat is derived from medium chain triglycerides. This therapy is the standard of care for LCHAD deficiency and other disorders of long-chain fatty acid …
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Tyni, T, Paetau A, Strauss AW, Middleton B, Kivela T (2004) Mitochondrial fatty acid {beta}-oxidation in human eye and brain: implications for retinopathy of long-chain 3-hydroxyacyl-CoA ...
long chain fatty acids disorder

BIOCHEMICAL, CLINICAL AND MOLECULAR FINDINGS IN …
INDIVUIDUAL’S NAME has a diagnosis of long chain 3-hydroxy acyl-CoA dehydrogenase (LCHAD)/Trifunctional protein (TFP) deficiency. This is a defect of fatty acid oxidation characterized …
fatty acid deficiency

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Biosynthesis of long chain PUFA is started from oleic acid (18:1Δ9) (Yap & Chen 2001). As seen in the Table 1, decreasing of oleic acid consentration is in line with the increasing of PUFA, implying that the oleic acid might act as the substrate for formation of the long chain fatty acid.
long chain fatty acid

Very Long Chain Acyl CoA Dehydrogenase Deficiency (LCAD) - NO…
Long-chain fatty acid oxidation disorders (FAODs) are a group of rare inherited inborn errors of metabolism. Individuals with long-chain FAODs are unable to use dietary fat (long chain fat) for energy. The inability to break down fats for energy and the buildup of fatty …
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[DOC File]Lipid and protein content for growth rate of Chaetoceros ...
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10. Takusa Y, Yamaguchi S. [Myopathies with miscellaneous disorders related to mitochondrial fatty acid oxidation: defective synthesis of ketone body, long-chain fatty acid transport defect, and muscular coenzyme Q10 deficiency…
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[DOC File]The Center for Inherited Disorders of Energy Metabolism ...
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Transport Of Long-Chain Fatty Acids (FA) Into Mitochondria. Carnitine has a key role in fat metabolism by transporting FA into the mitochondria for energy production. FA cannot permeate to the …
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[DOC File]Mitochondrial Dysfunction Brief: References
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The delta-5 and delta-6 desaturases involved in converting linoleic acid and alpha-linolenic acid to the longer chain polyunsaturates seem somewhat more sensitive to zinc deficiency than does the delta-9 desaturase converting stearic acid to oleic acid. "As a result, synthesis of the long chain polyunsaturates, especially arachidonic acid…
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[DOC File]What is Maryland’s Newborn Screening Program
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Fatty Acid Oxidation: Defects of fatty acid oxidation may be suspected in non-ketotic fasting hypoglycemia associated with dicarboxylic aciduria or in unexplained cardiomyopathy or rhabdomyolysis. Certain defects (e.g.: medium-chain acyl-CoA dehydrogenase deficiency…
long chain fatty acids list

[DOC File]Cunnane, 2005. Survival of the Fattest -- excerpts
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Very Long Chain Acyl-CoA Dehydrogenase Deficiency (VLCAD) Galactosemia (ga-LAK-toe-see-me-as) – The body cannot use a sugar in milk (galactose) because of the lack of an enzyme. A diet low in …
long chain fatty acids disorder

Long chain fatty acid deficiency