chain fatty acids and their derivatives results in neurologic dysfunction, which is seen as encephalopathy in these patients.[1] Causes of Phenotypic Variation MCADD is considered a "conformational disorder;" however, clinical manifestations of the disease span a broad spectrum of severity, even for...
Polyketides and Other Secondary Metabolites Including Fatty Acids and Their Derivatives AkihikoKawaguchi, ...NorihiroSato, inComprehensive Natural Products Chemistry, 1999 1.02.4.2Acyl-CoA Dehydrogenase and Acyl-CoA Oxidase The first reaction in the β-oxidation of straight-chain fatty acids i...
Likewise, the alpha-keto acids from catabolism of lysine and the BCAAs valine, leucine, and isoleucine in mitochondria can be conjugated to carnitine. These and various other organic acids are exported as short-chain acylcarnitines into circulation (Ji et al., 1987; Bremer and Hokland,...
α, ω- dicarboxylic acid, a method of producing ω- amino fatty acids or alcohols, methods of ester groups in the chain from the keto fatty acids to produce the introduced fatty acid derivatives by using the BVMO, and novel ω- hydroxy fatty acid which is produced by the above method....
Medium-Chain Acyl-CoA Dehydrogenase Deficiency in Children with Non-Ketotic Hypoglycemia and Low Carnitine Levels CHARLES A. STANLEY,'34' DANIEL E. HALE, PAUL M. COATES, CAROLE L. HALL, BARBARA E. CORKEY, WILLIAM YANG, RICHARD I. KELLEY, ELISA L. GONZALES, JOHN R. WILLIAMSON, AND LESTER...
The profile of organic acids in plasma of patients with a deficiency of medium-chain acyl-CoA dehydrogenase (EC 1.3.99.3) was determined by gas-liquid chromatography of trimethylsilylated derivatives of the acids isolated by ethyl acetat... M Duran,L Bruinvis,D Ketting,... - 《Clinical Chem...
Nyhan WLOzand PT Medium chain acyl CoA dehydrogenase deficiency. In:Atlas of Metabolic Diseases.London, England: Chapman & Hall; 1998:223-228. 2. Hoffmann GAramaki SBlum-Hoffmann ENyhan WLSweetman L Quantitative analysis for organic acids in biological samples: batch isolation followed by gas ...
However, the diagnosis may be missed by routine GC/MS of urinary organic acids in the asymptomatic state unless a poten- tially dangerous fasting test is performed (1). Besides enzymatic (2, 3) and molecular (4) analysis, two established biochemical methods are frequently used to diagnose ...
(e) Levels of ketone bodies can be easily measured in urine or blood by commercially available products (i.e. Ketostix®, Bayer, Inc.). Accordingly, the reader will see that the use of medium chain triglycerides (MCT) or fatty acids as a treatment and preventative measure of Alzheimer...
The ß-oxidation of fatty acids constitutes a repeated sequence of four reactions catalyzed by acyl-CoA dehydrogenases (ACADVL, ACADL, ACADM, ACADS), 2-enoyl-CoA hydratases (EHs), 3-hydroxy acyl-CoA dehydro- genases32, and 3-keto acyl-CoA thiolases (KATs), each of which comprise 2–4...