Author/Authors
Gerbert A. Jansen، نويسنده , , Nanda M. Verhoeven، نويسنده , , Simone Denis، نويسنده , , Gerrit-Jan Romeijn، نويسنده , , Cornelis Jakobs، نويسنده , , Herman J. ten Brink، نويسنده , , Ronald J. A. Wanders، نويسنده ,
DocumentNumber
1601490
Title Of Article
Phytanic acid α-oxidation: identification of 2-hydroxyphytanoyl-CoA lyase in rat liver and its localisation in peroxisomes
شماره ركورد
11713
Latin Abstract
Phytanic acid is broken down by α-oxidation in three steps finally yielding pristanic acid. The first step occurs in peroxisomes and is catalysed by phytanoyl-CoA hydroxylase. We have studied the second step in the α-oxidation pathway, which involves conversion of 2-hydroxyphytanoyl-CoA to pristanal catalysed by 2-hydroxyphytanoyl-CoA lyase. To this end, we have developed a stable isotope dilution gas chromatography-mass spectrometry assay allowing activity measurements in rat liver homogenates. Cell fractionation studies demonstrate that in rat liver 2-hydroxyphytanoyl-CoA lyase is localised in peroxisomes. This finding may have important implications for inherited diseases in man characterised by impaired phytanic acid α-oxidation.
From Page
176
NaturalLanguageKeyword
Phytanic acid , K-Oxidation , Pristanal , Pristanic acid , 2-Hydroxyphytanoyl-CoA lyase , Peroxisome
JournalTitle
Studia Iranica
To Page
182
To Page
182
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