Literature DB >> 12633678

Phytanic acid alpha-oxidation, new insights into an old problem: a review.

Ronald J A Wanders1, Gerbert A Jansen, Matthew D Lloyd.   

Abstract

Phytanic acid (3,7,10,14-tetramethylhexadecanoic acid) is a branched-chain fatty acid which is known to accumulate in a number of different genetic diseases including Refsum disease. Due to the presence of a methyl-group at the 3-position, phytanic acid and other 3-methyl fatty acids can not undergo beta-oxidation but are first subjected to fatty acid alpha-oxidation in which the terminal carboxyl-group is released as CO(2). The mechanism of alpha-oxidation has long remained obscure but has been resolved in recent years. Furthermore, peroxisomes have been found to play an indispensable role in fatty acid alpha-oxidation, and the complete alpha-oxidation machinery is probably localized in peroxisomes. This Review describes the current state of knowledge about fatty acid alpha-oxidation in mammals with particular emphasis on the mechanism involved and the enzymology of the pathway.

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Year:  2003        PMID： 12633678     DOI： 10.1016/s1388-1981(03)00003-9

Source DB:  PubMed          Journal:  Biochim Biophys Acta        ISSN： 0006-3002


  14 in total

Review 1.  Fatty acid 2-Hydroxylation in mammalian sphingolipid biology.

Authors:  Hiroko Hama
Journal:  Biochim Biophys Acta       Date:  2009-12-21

Review 2.  The functions of cardiolipin in cellular metabolism-potential modifiers of the Barth syndrome phenotype.

Authors:  Vaishnavi Raja; Miriam L Greenberg
Journal:  Chem Phys Lipids       Date:  2014-01-17       Impact factor: 3.329

3.  The critical role of Arabidopsis electron-transfer flavoprotein:ubiquinone oxidoreductase during dark-induced starvation.

Authors:  Kimitsune Ishizaki; Tony R Larson; Nicolas Schauer; Alisdair R Fernie; Ian A Graham; Christopher J Leaver
Journal:  Plant Cell       Date:  2005-07-29       Impact factor: 11.277

4.  Phytanic acid disturbs mitochondrial homeostasis in heart of young rats: a possible pathomechanism of cardiomyopathy in Refsum disease.

Authors:  Mateus Grings; Anelise Miotti Tonin; Lisiane Aurélio Knebel; Angela Zanatta; Alana Pimentel Moura; Carlos Severo Dutra Filho; Moacir Wajner; Guilhian Leipnitz
Journal:  Mol Cell Biochem       Date:  2012-04-15       Impact factor: 3.396

5.  The enzymology of mitochondrial fatty acid beta-oxidation and its application to follow-up analysis of positive neonatal screening results.

Authors:  Ronald J A Wanders; Jos P N Ruiter; Lodewijk IJLst; Hans R Waterham; Sander M Houten
Journal:  J Inherit Metab Dis       Date:  2010-05-20       Impact factor: 4.982

6.  Analysis of a range of catabolic mutants provides evidence that phytanoyl-coenzyme A does not act as a substrate of the electron-transfer flavoprotein/electron-transfer flavoprotein:ubiquinone oxidoreductase complex in Arabidopsis during dark-induced senescence.

Authors:  Wagner L Araújo; Kimitsune Ishizaki; Adriano Nunes-Nesi; Takayuki Tohge; Tony R Larson; Ina Krahnert; Ilse Balbo; Sandra Witt; Peter Dörmann; Ian A Graham; Christopher J Leaver; Alisdair R Fernie
Journal:  Plant Physiol       Date:  2011-07-25       Impact factor: 8.340

7.  Stereospecificity of fatty acid 2-hydroxylase and differential functions of 2-hydroxy fatty acid enantiomers.

Authors:  Lin Guo; Xu Zhang; Dequan Zhou; Adewole L Okunade; Xiong Su
Journal:  J Lipid Res       Date:  2012-04-19       Impact factor: 5.922

8.  Fatty acid 2-hydroxylase mediates diffusional mobility of Raft-associated lipids, GLUT4 level, and lipogenesis in 3T3-L1 adipocytes.

Authors:  Lin Guo; Dequan Zhou; Kenneth M Pryse; Adewole L Okunade; Xiong Su
Journal:  J Biol Chem       Date:  2010-06-02       Impact factor: 5.157

9.  In brain mitochondria the branched-chain fatty acid phytanic acid impairs energy transduction and sensitizes for permeability transition.

Authors:  Peter Schönfeld; Stefan Kahlert; Georg Reiser
Journal:  Biochem J       Date:  2004-10-01       Impact factor: 3.857

10.  Crystallization and preliminary X-ray crystallographic studies of fatty acid-CoA racemase from Mycobacterium tuberculosis H37Rv.

Authors:  Kyung Hee Rhee; Ki Seog Lee; Amit Priyadarshi; Eunice Eunkyung Kim; Kwang Yeon Hwang
Journal:  Acta Crystallogr Sect F Struct Biol Cryst Commun       Date:  2005-10-28
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