Phytanic acid alpha-oxidation: identification of 2-hydroxyphytanoyl-CoA lyase in rat liver and its localisation in peroxisomes
| Autor: | C.A.J.M. Jakobs, R. J. A. Wanders, Simone Denis, N. M. Verhoeven, G.-J. Romeijn, Gerrit Jansen, H. J. ten Brink |
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| Přispěvatelé: | Other departments |
| Jazyk: | angličtina |
| Rok vydání: | 1999 |
| Předmět: |
Male
Pristanic acid Phytanic acid 2-hydroxyphytanoyl-CoA lyase Biology Cell Fractionation Gas Chromatography-Mass Spectrometry chemistry.chemical_compound Peroxisomes Alpha oxidation Animals Carbon-Carbon Lyases Rats Wistar Molecular Biology Aldehydes Fatty Acids Cell Biology Peroxisome Lyase Rats Phytanic Acid Liver chemistry Biochemistry Rat liver Cell fractionation Oxidation-Reduction |
| Zdroj: | BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS, 1440(2-3), 176-182. Elsevier |
| ISSN: | 1388-1981 |
| Popis: | 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 alpha-oxidation. |
| Databáze: | OpenAIRE |
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