Fatty alpha-oxidation

Alpha oxidation and omega oxidation. Animal tissues degrade such straight-chain fatty acids as palmitic acid, stearic acid, and oleic acid almost entirely by (3 oxidation, but plant cells often oxidize fatty acids one carbon at a time. The initial attack may involve hydroxylation on the a-carbon atom (Eq. 17-3) to form either the d- or the L-2-hydroxy add.17 18-32 323 The L-hydroxy acids are oxidized rapidly, perhaps by dehydrogenation to the oxo acids (Eq. 17-3, step b) and oxidative decarboxylation, possibly utilizing H202 (see Eq. 15-36). The D-hydroxy acids tend to accumulate... 

Hashimoto T, Cook WS, Qi C, Yeldandi AV, Reddy JK, Rao MS. Defect in peroxisome proliferator-activated receptor alpha-inducible fatty acid oxidation determines the severity of hepatic steatosis in response to fasting. J Biol Chem 2000 275 28918-28928. 

A peroxisome proliferator is a chemical that induces peroxisome proliferation in rodent liver and other tissues and includes a wide range of chemicals such as certain herbicides, plasticizers, drugs, and natural products [38,39], The peroxisomes contain hydrogen peroxide and fatty acid oxidation systems important in lipid metabolism and activation of the peroxisome proliferator-activated receptor alpha (PPARa), is considered a key event in peroxisome proliferation in rodent hepatocytes [39], A number of studies have identified... 

Eldjarn L, Stokke O, Try K. 1966. Alpha-oxidation of branched chain fatty acids in man and its failure in patients with Refsum s disease showing phytanic acid accumulation. Scand J Clin Lab Invest 18 694-695. 

Leone, T. C., Weinheimer, C. J., and Kelly, D. P. A critical role for the peroxisome proliferator-activated receptor alpha (PPARalpha) in the cellular fasting response the PPARalpha-null mouse as a model of fatty acid oxidation disorders. Proc Natl Acad Sci U S A 96 (1999) 7473-7478. 

Vega, R. B., Huss, J. M., and Kelly, D. P. The coactivator PGC-1 cooperates with peroxisome prohferator-activated receptor alpha in transcriptional control of nuclear genes encoding mitochondrial fatty acid oxidation enzymes. Mol Cell Biol 20 (2000) 1868-1876. 

FATTY ACID ALPHA-OXIDATION ENZYMOLOGY AND DEFICIENCIES IN MAN... 

Croes, K., Casteels, M, de Hoffmann, E., Mannaerts,GP. Van Veldhoven,PP. (1996) r. J. Biochem. 240, 674 83. alpha-Oxidation of 3-methyl-substituted fatty acids in rat liver. Production of formic acid instead of C02, cofactor requirements, subcellular localization and formation of a 2-hydroxy-3-methylacyl-CoA intermediate. 

Croes, K., Casteels, M., Asselberghs, S., Herdewijn, P, Marmaerts, GP. Vanveldhoven, P.P. (1997) FEBS. Lett. 412, 643-645. Formation of a 2-methyl-branched fatty aldehyde during peroxisomal alpha-oxidation. 

Poulos, A., Sharp, R, Singh, H., Johnson, D.W., Carey, W.E. Easton, C. (1993) Biochem. J. 292, 457 61. Formic acid is a product of the alpha-oxidation of fatty acids by human skin fibroblasts deficiency of formic add production in peroxisome-deficient fibroblasts. 

PHYH Phytanoyl-CoA hydroxylase (Refsum disease) Lipid metabolism/fatty acid alpha-oxidation... 

Mead, J.F., Levis, G.M. Alpha oxidation of the brain fatty acids. Biochem. biophys. Res. Commun. 9, 231-234 (1962)... 

Alpha-oxidation of fatty acids has also been reported in plants, a process which involves sequential removal of one carbon at a time from free fatty acids of chain length ranging from C13 to Cig- It is unlikely that complete oxidation of fatty acids occurs in this manner, and the physiological significance of this pathway is obscure. It could well serve to shorten odd-chain fatty acids to even lengths and thus allow their degradation by jS-oxidation. 

The commonest polymethyl-branched fatty acid is probably phytanic or 3,7,11,15-tetramethylhexa-decanoic acid, which is a metabolite of phytol, and can be found in trace amounts in many animal tissues. It becomes a major component of the plasma lipids in Refsum s syndrome, a rare condition in which there is a deficiency in the enzymatic fatty acid alpha-oxidation system. Lough [562] has reviewed the occurrence and biochemistry of this and other isoprenoid fatty acids. Similar fatty acids are present in the lipids of the preen gland of birds and in those of tubercle bacilli. 

Inborn errors of metabolism Wilson s disease Alpha-l-antitrypsin deficiency Glycogen storage disease Tyrosinaemia Galactosaemia Cystic fibrosis Defects of fatty acid oxidation Other... 

Linear alpha-olefins are the source of the largest volume of ahphatic amine oxides. The olefin reacts with hydrogen bromide in the presence of peroxide catalyst, to yield primary alkyl bromide, which then reacts with dimethylamine to yield the corresponding alkyl dimethyl amine. Fatty alcohols and fatty acids are also used to produce amine oxides (Fig. 1). 

Lopez-Bote C.J., Sanz Arias R., Rey A.I, Castano A., Isabel B. and Thos J. (1998). Effect of free-range feeding on omega-3 fatty acids and alpha tocopherol content and oxidative... 

H. Tuunanen, J. Kuusisto, J. Toikka, P. Jaaskelainen, P. Maijamaki, K. Peuhkurinen, T. Viljanen, P. Sipola, K.Q. Stolen, J. Hannukainen, P. Nuutila, M. Laakso, J. Knuuti, Myocardial perfusion, oxidative metabolism, and free fatty acid uptake in patients with hypertrophic cardiomyopathy attributable to the Asp175Asn mutation in the alpha-tropomyosin gene A positron emission tomography study, J. Nucl. Cardiol. 14(2007) 354-365. 

Yamauchi, K., Nagai, Y., and Ohashi, T. 1982. Quantitative relationship between alpha-tocopherol and polyunsaturated fatty acids and its connection to development of oxidative rancidity in chicken skeletal muscle. Agric. Biol. Chem. 46 2719-2724. 

In fractions of butteroil containing alpha-tocopherol and copper. These workers reported that l,5-octadlen-3-one from the oxidation of long-chain polyunsaturated n-3 fatty acids was responsible for metallic notes, but the fishiness appeared to be contributed by the 2,A,7-decatrlenals or a combination of these compounds. This relationship has been explored further In our laboratory through mechanistic studies employing alpha-tocopherol and Trolox C which Is a synthetic tocopherol-type antioxidant (Figure 2). 

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