Fatty acids mitochondrial

Bennett, M. J., 1994. The enzyme.s of mitochondrial fatty acid oxidation. 

Mitochondrial Oxidations Mitochondrial Fatty Acid Oxidation... 

Figure 3. Mitochondrial fatty acid oxidation. Long-chain fatty acids are converted to their CoA-esters as described in the text, and their fatty-acyl-groups transferred to CoA in the matrix by the concerted action of CPT 1, the acylcarnitine/carnitine exchange carrier and CPT (A) as described in the text. Medium-chain and short-chain fatty acids (Cg or less) diffuse directly into the matrix where they are converted to their acyl-CoA esters by a acyl-CoA synthase. The mechanism of p-oxidation is shown below (B). Each cycle of P-oxidation removes -CH2-CH2- as an acetyl unit until the fatty acids are completely converted to acetyl-CoA. The enzymes catalyzing each stage of P-oxidation have different but overlapping specificities. In muscle mitochondria, most acetyl-CoA is oxidized to CO2 and H2O by the citrate cycle (Figure 4) some is converted to acylcamitine by carnitine acetyltransferase (associated with the inner face of the inner membrane) and exported from the matrix. Some acetyl-CoA (if in excess) is hydrolyzed to acetate and CoASH by acetyl-CoA hydrolase in the matrix. Enzymes ...

The carnitine shuttle is the rate-determining step in mitochondrial fatty acid degradation. Malonyl CoA, a precursor of fatty acid biosynthesis, inhibits carnitine acyltransferase (see p. 162), and therefore also inhibits uptake of fatty acids into the mitochondrial matrix. 

Toxicity assessment. Ethanol extract of the leaf, administered intraperitoneally to mice, was active, LDjf, 0.75 g/kg"" " . Ethanol extract of the fresh leaf and stem, administered intraperitoneally to mice at the minimum toxic dose of 1 mL/animal, was active. Water extract of the fresh leaf and stem, administered intraperitoneally to mice at the minimum toxic dose of 1 mL/ animal, was active " . Aqueous extract of the husk fiber, administered orally to mice, was active, LDjf, 2.30 g/kgf" " . Tricarboxylate carrier influence. Oil, administered to rats at a dose of 15% of the diet for 3 weeks, produced a differential mitochondrial fatty acid composition and no appreciable change in phospholipids composition and cholesterol level. Compared with coconut oil-fed rats, the mitochondrial tricarboxylate carrier activity was markedly decreased in liver mitochondria from fish oil-fed rats. No difference in the Arrhenius plot between the two groups was observed "". 

The 3-OH FAs have had great utility in the determination of LPS levels in indoor air. However, in tissues and body fluids it has been determined that 3-OH FAs are naturally present at low levels as products of mammalian metabolism (mitochondrial fatty acid p oxidation). Due to this background GC-MS/MS for 3-OH FAs is not recommended as a general marker to determine trace LPS levels in clinical samples [14]. However, in certain situations the assessment of 3-OH FAs has been successfully used, for example, in the diagnosis of chronic peridontitis [15]. There is great potential for the utility of 3-OH FAs as markers for LPS contamination in pharmaceutical products, where often the background matrix would be anticipated to be much less complex. 

Table 3.1.1 Disorders of organic acid metabolism (in alphabetical order). This table does not include disorders with primary accumulation of amino acids, disorders of mitochondrial fatty acid oxidation, or primary lactic acidemias. Co A Coenzyme A, FAD flavin adenine dinucleotide...

Hoffmann GP, von Kries R, Klose D, Lindner M, Schulze A, Muntau AC, Roschinger W, Liebl B, Mayatepek E, Roscher AA (2004) Prequencies of inherited organic acidurias and disorders of mitochondrial fatty acid transport and oxidation in Germany. Eur J Pediatr 163 76-80... 

Nada MA, Vianey-Saban C, Roe CR, et al (1996) Prenatal diagnosis of mitochondrial fatty acid oxidation defects. Prenat Diagn 16 117-124... 

Schmidt-Sommerfeld E, Bobrowski PJ, Penn D, Rhead WJ, Wanders RJA, Bennet MJ (1998) Analysis of carnitine esters by radio-high performance liquid chromatography in cultured skin fibroblasts from patients with mitochondrial fatty acid oxidation disorders. Pediatr Res 44 210-214... 

Giak Sim K, Carpenter K, Hammond J, Christodoulou J, Wilcken (2002) Quantitative fibroblast acylcarnitine profiles in mitochondrial fatty acid beta-oxidation defects phenotype/me-tabolite correlations. Mol Genet Metab 76 327-334... 

Glucagon exerts a ketogenic action on the liver which is more pronounced in insulin-deficient states. This action is thought to be due mainly to the inhibition of acetyl-CoA carboxylase with resulting decrease in malonyl-CoA. Malonyl-CoA is an inhibitor of carnitine acyltransferase I which is the rate-limiting step for mitochondrial fatty acid oxidation. A decrease in malonyl-CoA is thus postulated to lead to overproduction of acetyl-CoA which is then condensed to form ketone bodies. 

Infante JP (1999) A function for the vitaminE metabolite alpha-tocopherol quinone as an essential enzyme cofactor for the mitochondrial fatty acid desaturases. FEES letters 446, 1-5. 

Accumulation of microvesicular lipid droplets between myofibrils adjacent to mitochondria was found in muscle biopsies from seven children taking valproate (SEDA-22, 92). Ultrastructural abnormalities in the mitochondria suggested that these could have resulted from impaired mitochondrial fatty acid oxidation. 

Vega R, Fluss J, Kelly D. the coactivator PGC-1 cooperates with peroxisome proliferator-activated receptor a in transcriptional of nuclear genes encoding mitochondrial fatty acid oxidation enzymes. Mol Cell Biol 2000 20 1868-1876. 

Treem WR. Mitochondrial fatty acid oxidation and acute fatty liver of pregnancy. Semin Gastrointest Dis 2002 13 55-66. 

Jackson S, Kler RS, Bartlett K, Briggs H, Bindoff LA, Pomfarzam M, et al. Combined enzyme defect of mitochondrial fatty acid oxidation. J Clin Invest 1992 90 1219-25. 

Wanders RJ, Ijlst L, Poggi F, Bonnefont JP, Munnich A, Brivet M, et al. Human trifimctional protein deficiency a new disorder of mitochondrial fatty acid beta-oxidation. Biochem Biophys Res Commun 1992 188 1139-45. 

Mitochondrial fatty acid elongation occurs primarily when the [NADH]/[NAD+] ratio is high (e.g., anaerobiosis, excessive ethanol oxidation). 

Roc. C. R., and Coates, P, M. 1995. Mitochondrial fatty acid oxidation disorders. In The Metabolic Basis of Inherited Diseases (7th ed., pp. 

Tracers used for assessment of myocardial metabolism are selected based on the type of metabolism of interest EDO traces glucose metabolism, ["Cjpalmitate traces mitochondrial fatty acid metabolism, and ["Cjacetate is an indirect marker for myocardial oxygen consumption, allowing assessment of ventricular performance. [ CjPalmitate is a useful marker for normal myocardial oxygen consumption because baseline energy needs of the myocardium... 

Roe CR, Ding I. Mitochondrial fatty acid oxidation disorders. In Scriver CR, Beaudet AL, Sly WS, et al., eds. The Metabolic and Molecular Basis of Inherited Disease, 8th ed. New York McGraw-HiU, 2001 2297-2326. 

Hashimoto, T., Fujita, T., Usuda, N., Cook, W., Qi, C., Peters, J. M., Gonzalez, F. J., Yeldandi, A. V., Rao, M. S., and Reddy, J. K. (1999). Peroxisomal and mitochondrial fatty acid beta-oxidation in mice nullizygous for both peroxisome proUferator-activated receptor alpha and peroxisomal fatty acyl-CoA oxidase. Genotype correlation with fatty liver phenotype. J Biol Chem 274, 19228-19236. 

Gregersen N, Andresen BS, Corydon MJ, Corydon TJ, Olsen RK, Bolund L, Bross P. Mutation analysis in mitochondrial fatty acid oxidation defects Exemplified by acyl-CoA dehydrogenase deficiencies, with special focus on genotype-phenotype relationship. Hum Mutat. 2001 Sep 18(3) 169-89. Review. PubMed citation... 

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