Biosynthesis of glyceryl triphytanate in rabbit receiving a diet enriched with phytol

Sezille, G; Jaillard, J; Scherpereel, P; Biserte, G

doi:10.1016/0009-8981(70)90055-0

Cited by 5 publications

(1 citation statement)

References 8 publications

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“…For the triacylglycerols, this was mainly due to the presence of novel species with a distinctive TLC migration pattern (higher R f ) (Figure 4). Similar species have been reported in cardiac triacylglycerols of rabbits fed phytol (Sezille et al, 1970), and mice lacking HACL1 when exposed to phytol (Mezzar et al, 2017). GC analysis showed that both cholesteryl esters and triacylglycerols were enriched in phytanic and pristanic acid, although with a different ratio 8 .…”

Section: Challenging Of Adult Pmp34 Deficient Micesupporting

confidence: 77%

Slc25a17 Gene Trapped Mice: PMP34 Plays a Role in the Peroxisomal Degradation of Phytanic and Pristanic Acid

Veldhoven

Schryver

Young

et al. 2020

Front. Cell Dev. Biol.

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Mice lacking PMP34, a peroxisomal membrane transporter encoded by Slc25a17, did not manifest any obvious phenotype on a Swiss Webster genetic background, even with various treatments designed to unmask impaired peroxisomal functioning. Peroxisomal αand β-oxidation rates in PMP34 deficient fibroblasts or liver slices were not or only modestly affected and in bile, no abnormal bile acid intermediates were detected. Peroxisomal content of cofactors like CoA, ATP, NAD + , thiamine-pyrophosphate and pyridoxal-phosphate, based on direct or indirect data, appeared normal as were tissue plasmalogen and very long chain fatty acid levels. However, upon dietary phytol administration, the knockout mice displayed hepatomegaly, liver inflammation, and an induction of peroxisomal enzymes. This phenotype was partially mediated by PPARα. Hepatic triacylglycerols and cholesterylesters were elevated and both phytanic acid and pristanic acid accumulated in the liver lipids, in females to higher extent than in males. In addition, pristanic acid degradation products were detected, as wells as the CoAesters of all these branched fatty acids. Hence, PMP34 is important for the degradation of phytanic/pristanic acid and/or export of their metabolites. Whether this is caused by a shortage of peroxisomal CoA affecting the intraperoxisomal formation of pristanoyl-CoA (and perhaps of phytanoyl-CoA), or the SCPx-catalyzed thiolytic cleavage during pristanic acid β-oxidation, could not be proven in this model, but the phytol-derived acyl-CoA profile is compatible with the latter possibility. On the other hand, the normal functioning of other peroxisomal pathways, and especially bile acid formation, seems to exclude severe transport problems or a shortage of CoA, and other cofactors like FAD, NAD(P) + , TPP. Based on our findings, PMP34 deficiency in humans is unlikely to be a life threatening condition but could cause elevated phytanic/pristanic acid levels in older adults.

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Section: Challenging Of Adult Pmp34 Deficient Micesupporting

confidence: 77%