Literature DB >> 9266223

Stable isotope studies of phytanic acid alpha-oxidation: in vivo production of formic acid.

N M Verhoeven1, D S Schor, S F Previs, H Brunengraber, C Jakobs.   

Abstract

The aim of this study was to test whether formate is formed during alpha-oxidation of phytanic acid in humans. To a healthy volunteer, [1-13C]phytanic acid was given as an oral substrate in a dose of 15 mg/kg body weight, after which plasma, urine and breath air samples were collected during 35 h. The plasma concentrations of [1-13C]-phytanic acid, 2-hydroxy[1-13C]phytanic acid, pristanic acid and [13C]formate were analysed. The [1-13C]phytanic acid concentration increased within 5-7 h to 105 mumol/l, then decreased. Formation of 2-hydroxy[1-13C]phytanic acid increased during the first 11 h after which it decreased during the next 20 h. Pristanic acid increased slightly during the test. In breath air, 13CO2 enrichment was measured, showing a cumulative output of ca. 30% of the ingested dose after 35 h. In both urine and plasma, enrichment of [13C]formate, higher than that of 13CO2 was demonstrated. These findings show that formate is a decarboxylation product in the alpha-oxidation of phytanic acid in vivo.

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Year:  1997        PMID: 9266223     DOI: 10.1007/pl00014279

Source DB:  PubMed          Journal:  Eur J Pediatr        ISSN: 0340-6199            Impact factor:   3.183


  4 in total

1.  Formate can differentiate between hyperhomocysteinemia due to impaired remethylation and impaired transsulfuration.

Authors:  Simon G Lamarre; Anne M Molloy; Stacey N Reinke; Brian D Sykes; Margaret E Brosnan; John T Brosnan
Journal:  Am J Physiol Endocrinol Metab       Date:  2011-09-20       Impact factor: 4.310

2.  Intermediates and products formed during fatty acid alpha-oxidation in cucumber (Cucumis sativus).

Authors:  G I Borge; G Vogt; A Nilsson
Journal:  Lipids       Date:  1999-07       Impact factor: 1.880

3.  Transport of phytanic acid on lipoproteins in Refsum disease.

Authors:  A S Wierzbicki; A Sankaralingam; P J Lumb; T C Hardman; M C Sidey; F B Gibberd
Journal:  J Inherit Metab Dis       Date:  1999-02       Impact factor: 4.982

4.  Catabolism of 4-hydroxyacids and 4-hydroxynonenal via 4-hydroxy-4-phosphoacyl-CoAs.

Authors:  Guo-Fang Zhang; Rajan S Kombu; Takhar Kasumov; Yong Han; Sushabhan Sadhukhan; Jianye Zhang; Lawrence M Sayre; Dale Ray; K Michael Gibson; Vernon A Anderson; Gregory P Tochtrop; Henri Brunengraber
Journal:  J Biol Chem       Date:  2009-09-15       Impact factor: 5.157

  4 in total

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