Phytanic acid α-oxidation:: identification of 2-hydroxyphytanoyl-CoA lyase in rat liver and its localisation in peroxisomes

被引：25

作者：

Jansen, GA

Verhoeven, NM

Denis, S

Romeijn, GJ

Jakobs, C

ten Brink, HJ

Wanders, RJA

机构：

[1] Univ Amsterdam, Acad Med Ctr, Dept Pediat, Emma Childrens Hosp,Lab Genet Metab Dis, NL-1100 DE Amsterdam, Netherlands

[2] Free Univ Amsterdam Hosp, Dept Clin Chem, Metab Unit, Amsterdam, Netherlands

[3] Univ Amsterdam, Acad Med Ctr, Dept Clin Chem, Lab Genet Metab Dis, NL-1100 DE Amsterdam, Netherlands

来源：

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS | 1999年 / 1440卷 / 2-3期

关键词：

phytanic acid; alpha-oxidation; pristanal; pristanic acid; 2-hydroxyphytanoyl-CoA lyase; peroxisome;

D O I：

10.1016/S1388-1981(99)00126-2

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

Phytanic acid is broken down by alpha-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 alpha-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 cc-oxidation. (C) 1999 Elsevier Science B.V. All rights reserved.

引用

页码：176 / 182

页数：7

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