Phytanoyl‐CoA hydroxylase activity is induced by phytanic acid

Zomer, Anna W.M.; Jansen, G. A.; Burg, Bart van der; Verhoeven, Nanda M.; Jakobs, Cornelis; Saag, Paul T. van der; Wanders, Ronald J. A.; Poll-Thé, Bwee Tien

doi:10.1046/j.1432-1327.2000.01451.x

Cited by 27 publications

(27 citation statements)

References 30 publications

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“…Ostlund Farrants et al (1990), however, described induction of the branched-chain trihydroxicoprostanoylCoA oxidase by fatty diets in rodents. Phytanoyl-CoA hydroxylase activity was induced up to four times in cell lines grown in the presence of phytanic acid (Zomer et al 2000). In mouse liver, phytanic acid caused a differential induction of palmitate peroxisomal -oxidizing capacity (5-fold), of total catalase activity (only 1·5-fold) and urate or glycolate oxidase activities (no change).…”

Section: Discussionmentioning

confidence: 95%

Modulation of the peroxisomal gene expression pattern by dehydroepiandrosterone and vitamin D: therapeutic implications

Depreter

Vandesompele²,

Espeel³

et al. 2002

Journal of Endocrinology

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Peroxisomes are ubiquitous organelles required for several metabolic functions. Their dysfunction is responsible for a group of human inherited disorders. In the search for endogenous factors regulating the peroxisomal compartment in normal liver, we treated female rats with dehydroepiandrosterone (DHEA) and 25-hydroxycholecalciferol for 1 and 6 days. Relative transcription levels of 39 selected genes were evaluated by real-time quantitative RT-PCR analysis. Catalase (peroxisomal marker)-specific activity was assayed in total liver homogenate and peroxisomes were visualized by catalase localization. DHEA induced peroxisome proliferation and raised catalase specific activity. Expression levels of 16 (of which 11 were peroxisomal) genes were altered. Pex 11, acyl-CoA oxidase, -and -multifunctional enzyme, thiolase 1, phytanoyl-CoA hydroxylase, 70 kDa peroxisomal membrane protein and very long chain acyl-CoA synthetase were upregulated, three others were downregulated. Vitamin D caused downregulation of six genes. Administration of vitamin D to peroxisomal disorder patients may be contraindicated. The adrenocortical hormone DHEA is a potential natural regulator of the peroxisomal compartment. Its therapeutic use in X-linked adrenoleukodystrophy, some other -oxidation defects and classical Refsum should be considered.

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Section: Discussionmentioning

confidence: 95%

Modulation of the peroxisomal gene expression pattern by dehydroepiandrosterone and vitamin D: therapeutic implications

Depreter

Vandesompele²,

Espeel³

et al. 2002

Journal of Endocrinology

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“…The effect of elevated maternal phytanic acid on maternal or foetal outcomes in pregnancy has never been described. It has been postulated that phytanic acid does not cross the placenta (Zomer et al 2000) but clinical data is lacking. In this case it seems that elevated plasma phytanic acid that was not detrimental to the mother or foetus.…”

Section: Discussionmentioning

confidence: 99%

The Challenges of a Successful Pregnancy in a Patient with Adult Refsum’s Disease due to Phytanoyl-CoA Hydroxylase Deficiency

et al. 2016

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We describe the management and outcomes of pregnancy in a 27-year-old woman with infantile-onset Adult Refsum's disease (ARD). She presented in infancy but was diagnosed with ARD at the age of 10 on basis of phytanic acidaemia and later confirmed to have the phytanoyl-CoA hydroxylase ((PHYH) c.164delT, p. L55fsX12) mutation. Despite repeated plasmapheresis sessions and strict dietary surveillance for 20 years, her phytanic acid levels persistently stayed above the ideal target level of 100 mmol/L but remained below 400 mmol/L. Initially the pregnancy was uncomplicated but in the third trimester of pregnancy the patient was admitted to the hospital with fluctuating hypertension, sinus tachycardia and breathlessness. The patient was compliant with diet during pregnancy and her phytanic levels were remained well controlled: 177 and 188 mmol/L in the first and second trimester, respectively. Peri-partum management required a coordinated team approach including a high-calorie and restricted diet to reduce the risk of acute metabolic decompensation. During the induced labour she required 10% dextrose infusions.Post-partum it took the mother a long time to recover from childbirth -her appetite was poor due to post-natal depression and her body weight decreased rapidly by 11 kg within 3 weeks after childbirth, resulting in a spike in phytanic acid to 366 mmol/L. Measures were taken to minimise the risk of acute neurological decompensation. The infant was unaffected and has made normal developmental progress in the subsequent 2 years.

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“…Phytanic acid (3,7,11,15-tetramethylhexadecanoic acid) is a methyl-branched fatty acid that originates from the phytol side of the chain of chlorophyll. Phytanic acid enters the human body in foods such as dairy products or other ruminant fats.…”

Section: Introductionmentioning

confidence: 99%

“…Pristanic acid can also activate PPARK [7]. We have recently reported that phytanic acid, when added to cell culture medium, is an activator of brown adipocyte di¡erentiation and transcription of the uncoupling protein-1 (UCP1) gene, the speci¢c marker of brown adipocytes [8].…”

Section: Introductionmentioning

confidence: 99%

Phytanic acid, but not pristanic acid, mediates the positive effects of phytol derivatives on brown adipocyte differentiation

et al. 2002

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The phytol derivatives phytanic acid and pristanic acid may activate nuclear hormone receptors and influence gene expression and cell differentiation. Phytanic acid induces brown adipocyte differentiation. It was determined that brown fat and brown adipocytes are sites of high gene expression of phytanoylCoA hydroxylase, the enzyme required for initiation of peroxisomal K K-oxidation of phytanic acid. However, the effects of phytanic acid were not mediated by its K K-oxidation product pristanic acid, which did not promote brown adipocyte differentiation or stimulate transcription of the uncoupling protein-1 gene. Moreover, acute cold exposure of mice caused a dramatic mobilization of the phytanic acid stores in brown adipose tissue thus suggesting that a high local exposure to phytanic acid in brown fat may contribute to signalling adaptive changes in the tissue in response to thermogenic activation. ß

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Phytanoyl‐CoA hydroxylase activity is induced by phytanic acid

Cited by 27 publications

References 30 publications

Modulation of the peroxisomal gene expression pattern by dehydroepiandrosterone and vitamin D: therapeutic implications

Modulation of the peroxisomal gene expression pattern by dehydroepiandrosterone and vitamin D: therapeutic implications

The Challenges of a Successful Pregnancy in a Patient with Adult Refsum’s Disease due to Phytanoyl-CoA Hydroxylase Deficiency

Phytanic acid, but not pristanic acid, mediates the positive effects of phytol derivatives on brown adipocyte differentiation

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