Minor constitutents of human milk IV: Analysis of the branched chain fatty acids

Egge, Heinz; Murawski, U.; Ryhage, Ragnar; György, Paul; Chatranon, Wirapong; Zilliken, F.

doi:10.1016/0009-3084(72)90042-4

Cited by 43 publications

(36 citation statements)

References 27 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%

“…trimethyltridecanoic acid, is the presumed breakdown product of the 13-oxidation of pristanic acid. It, too, is a normal dietary constituent [14] and has been reported to be present in human milk [7]. Whether its apparent accumulation in some patients with peroxisomal disease is secondary to the large increase in pristanic acid concentration in those individuals or whether its presence is indicative of their inability to oxidise this fatty acid is not known but clearly warrants further study.…”

Section: Discussionmentioning

confidence: 97%

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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%

Section: Discussionmentioning

confidence: 97%

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 breastfed infant consumes BCFA since they are found in colostrum and mature milk at concentrations up to 1.5% w/w [23]. Taking 0.6% as a conservative estimate of mean BCFA concentration in American human milk [24] with 3.2%fat, we estimate that consumption of BCFA is about 19 mg BCFA per 100 mL human milk.…”

Section: Bcfa Intake In Infants and Adultsmentioning

confidence: 99%

Branched-Chain Fatty Acids in the Neonatal Gut and Estimated Dietary Intake in Infancy and Adulthood

Ran-Ressler

Glahn

Bae

et al. 2013

Nestlé Nutrition Institute Workshop Series

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Branched chain fatty acids (BCFA) are primarily saturated fatty acids (FA) with a methyl branch, usually near the terminal methyl group. BCFA are abundant in bacteria, skin, and vernix caseosa but have seldom been studied with respect to human nutrition. They are constituents of the term newborn infant gut lumen, being swallowed as vernix particulate components of amniotic fluid in the last trimester of normal pregnancy. We recently showed that BCFA protect against Necrotizing Enterocolitis (NEC) in the rat pup model. Dietary BCFA at levels similar to those found in human vernix reduced NEC incidence by more than 50%, increased the abundance of BCFA-containing bacteria, and increased the expression of ileal anti-inflammatory IL-10. The few published reports of BCFA in human milk enable an estimate that breast fed infants consume 19 mg BCFA per 100ml milk. Dietary BCFA consumption from milkfat and other ruminant products, the main sources of dietary BCFA, is more than 400 mg BCFA per day in adult Americans. This estimate exceeds by several fold the average dietary intake of bioactive FA, such as docosahexaenoic acid. BCFA are bioactive, abundant but neglected components of the human food supply.

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“…The latter compound can be generated from 5,9,13-trimethyltetradecanoic acid (which is also present in human milk) by one cycle of ␤-oxidation but can also be derived from farnesoic acid, inasmuch as in the work-up of the samples, all double bonds were reduced. In addition, 3-and 5-methyl mono-branched medium-chain fatty acids were also shown to be constituents of the lipid fraction of human milk (27).…”

Section: Activity Of Pahx Depends On the Chain Length Of The Branch Amentioning

confidence: 99%

“…However, given the prevalence in nature of isoprenoids as geraniol and farnesol, such 3-methyl-branched mediumchain fatty acids are more than likely present in the diet of man. In a thorough analysis of human milk, traces of 3,7,11-trimethyldodecanoic acid were found (27). The latter compound can be generated from 5,9,13-trimethyltetradecanoic acid (which is also present in human milk) by one cycle of ␤-oxidation but can also be derived from farnesoic acid, inasmuch as in the work-up of the samples, all double bonds were reduced.…”

Section: Activity Of Pahx Depends On the Chain Length Of The Branch Amentioning

confidence: 99%

Further studies on the substrate spectrum of phytanoyl-CoA hydroxylase

Foulon

Asselberghs

Geens

et al. 2003

Journal of Lipid Research

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Minor constitutents of human milk IV: Analysis of the branched chain fatty acids

Cited by 43 publications

References 27 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

Branched-Chain Fatty Acids in the Neonatal Gut and Estimated Dietary Intake in Infancy and Adulthood

Further studies on the substrate spectrum of phytanoyl-CoA hydroxylase

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