The isolation and identification of 2,6,10,14-tetramethylpentadecanoic acid from butterfat

Hansen, R. P.; Morrison, J. W.

doi:10.1042/bj0930225

Cited by 43 publications

(8 citation statements)

References 12 publications

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“…It has been speculated that isoprenoid fatty acids such as phytanic and pristanic acids are not synthesised de novo in humans but are dietary constituents [32] and this is the probable source of the pristanic acid which accumulates in these various patients [7,10,14]. The marked reduction in pristanic acid levels we observed in one patient after the introduction of a low phytanic acid diet, a diet which is probably low in pristanic acid as well [2,14], provides strong evidence to support this view.…”

Section: Discussionsupporting

confidence: 66%

Accumulation of pristanic acid (2, 6, 10, 14 tetramethylpentadecanoic acid) in the plasma of patients with generalised peroxisomal dysfunction

et al. 1988

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The plasma of some patients with biochemical evidence of a generalised peroxisomal dysfunction (GPD) show greatly increased levels of phytanic acid as well as its alpha-oxidation product, pristanic acid (2, 6, 10, 14-tetramethylpentadecanoic acid). Increased amounts of 14- and 16- carbon branched chain fatty acids are also found in some of these patients. As pristanic acid is present in normal or near-normal amounts in classical Refsum disease and rhizomelic chondrodysplasia, two disorders characterised by deficiencies in phytanic acid oxidation, we speculate that its accumulation is not secondary to a defect in the alpha-oxidation of phytanic acid, but is indicative of a block in the peroxisomal beta-oxidation of pristanic acid. The finding of phytanic acid, as well as a number of its metabolites in patients with inherited defects in peroxisomal biogenesis indicates that a number of the steps in phytanic acid degradation may be confined to peroxisomes.

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Section: Discussionsupporting

confidence: 66%

Accumulation of pristanic acid (2, 6, 10, 14 tetramethylpentadecanoic acid) in the plasma of patients with generalised peroxisomal dysfunction

et al. 1988

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“…Dans la matière grasse du lait de bufflonne les proportions respectives des acides 4, 8, 12-triméthyltridécanoïque, pristanique et phytanique et indiqués dans le tableau 3 sont exactement similaires aux proportions de ces mêmes acides déterminés dans la matière grasse du lait de vache [22]. Dans la matière grasse du lait de bufflonne la proportion d'acide phytanique est environ dix fois plus élevée que la proportion de l'acide 4,8, 12-triméthyltridécanoïque; ceci est également observé dans le cas de la matière grasse du lait de vache [17,38].…”

Section: Etude Comparée Des Proportions Relatives Des Acides Gras Danunclassified

Contribution à l'étude des composés liposolubles du lait de bufflonne

Addeo

Kuzdzal-Savoie

Chianese

et al. 1981

Lait

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“…The resulting pristanic acid (2,6,10,14-tetramethylpentadecanoic acid) is then further degraded by /?-oxidation, yielding propionyl-CoA and acetyl-CoA. Some pristanic acid is also derived directly from dietary sources (Hansen and Morrison, 1964).…”

mentioning

confidence: 99%

Purification and properties of an α‐methylacyl‐CoA racemase from rat liver

Schmitz

Fingerhut

Conzelmann

1994

European Journal of Biochemistry

105

134

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The (R)-and (S)-isomers of a-methyl-branched fatty acids were shown to be rapidly interconverted as coenzyme A thioesters, by an a-methylacyl-CoA racemase. The enzyme was purified some 5600-fold from rat liver, to apparent homogeneity. It is a monomer of 45 kDa with an isolectric point of pH 6.1 and is optimally active between pH 6 and pH 7. It acts only on coenzyme A thioesters, not on free fatty acids, and accepts as substrates a wide range of a-methylacyl-CoAs, including pristanoyl-CoA and trihydroxycoprostanoyl-CoA (an intermediate in bile acid synthesis), but neither 3-methyl-branched nor linear-chain acyl-CoAs. The racemase catalyzes a rapid exchange of the H atom in the a-position of the fatty acid against a proton from water, indicating that the mechanism involves abstraction of a proton. Based on this observation, a very sensitive and convenient radiometric assay, with 2-methy1[2-3H]acyl-CoAs as substrates, was developed. The enzyme was inactivated by micromolar concentrations of Hg2' and to a lesser extent by Cu2+ but not by iodoacetamide and only slightly by N-ethylmaleimide and thimerosal.

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The isolation and identification of 2,6,10,14-tetramethylpentadecanoic acid from butterfat

Abstract: Butterfat has been shown to contain small amounts of branched-chain fatty acids of the iso and anteiso series (Hansen & Shorland, 1951a;

Cited by 43 publications

References 12 publications

Accumulation of pristanic acid (2, 6, 10, 14 tetramethylpentadecanoic acid) in the plasma of patients with generalised peroxisomal dysfunction

Accumulation of pristanic acid (2, 6, 10, 14 tetramethylpentadecanoic acid) in the plasma of patients with generalised peroxisomal dysfunction

Contribution à l'étude des composés liposolubles du lait de bufflonne

Purification and properties of an α‐methylacyl‐CoA racemase from rat liver

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