Stable isotope studies of phytanic acid alpha-oxidation: in vivo production of formic acid.
Eur J Pediatr
; 156 Suppl 1: S83-7, 1997 Aug.
Article
em En
| MEDLINE
| ID: mdl-9266223
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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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Ácido Fitânico
/
Formiatos
Limite:
Adult
/
Female
/
Humans
Idioma:
En
Revista:
Eur J Pediatr
Ano de publicação:
1997
Tipo de documento:
Article