Literature DB >> 8386940

Studies on phytanic acid alpha-oxidation in rat liver and cultured human skin fibroblasts.

R J Wanders1, C W Van Roermund.   

Abstract

We have studied the alpha-oxidation of phytanic acid in rat liver and human skin fibroblasts in order to try to resolve the controversial issue of the subcellular site of alpha-oxidation of phytanic acid. The results show that isolated mitochondria are able to alpha-oxidize phytanic acid whereas isolated peroxisomes show no phytanic acid alpha-oxidation activity. Intact hepatocytes were found to alpha-oxidize phytanic acid at a rate which is more than 20-fold higher than the activity found in postnuclear supernatant fractions incubated under optimal conditions. The alpha-oxidation of phytanic acid was found to be sensitive to inhibitors of the respiratory chain and an uncoupler of oxidative phosphorylation. Furthermore, the alpha-oxidation of phytanic acid was found to be deficient in cultured human skin fibroblasts with an inherited deficiency of cytochrome c oxidase and in fibroblasts with a deficiency of functional peroxisomes. We conclude that mitochondria are indispensable for phytanic acid alpha-oxidation. Furthermore, we propose that one (or more) of the partial reactions in phytanic acid alpha-oxidation proceeds in peroxisomes leading to the concept that phytanic acid oxidation in the intact cell requires the participation of both mitochondria and peroxisomes.

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Year:  1993        PMID： 8386940     DOI： 10.1016/0005-2760(93)90239-6

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


  12 in total

1.  Peroxisomal localization of alpha-oxidation in human liver.

Authors:  M Casteels; K Croes; P P Van Veldhoven; G P Mannaerts
Journal:  J Inherit Metab Dis       Date:  1997-09       Impact factor: 4.982

2.  A novel PEX12 mutation identified as the cause of a peroxisomal biogenesis disorder with mild clinical phenotype, mild biochemical abnormalities in fibroblasts and a mosaic catalase immunofluorescence pattern, even at 40 degrees C.

Authors:  Avraham Zeharia; Merel S Ebberink; Ronald J A Wanders; Hans R Waterham; Alisa Gutman; Andreea Nissenkorn; Stanley H Korman
Journal:  J Hum Genet       Date:  2007-05-30       Impact factor: 3.172

3.  Phytanic acid oxidation in man: identification of a new enzyme catalysing the formation of 2-ketophytanic acid from 2-hydroxyphytanic acid and its deficiency in the Zellweger syndrome.

Authors:  R J Wanders; C W van Roermund; D S Schor; H J ten Brink; C Jakobs
Journal:  J Inherit Metab Dis       Date:  1995       Impact factor: 4.982

4.  Defective kinetics of cytochrome c oxidase and alteration of mitochondrial membrane potential in fibroblasts and cytoplasmic hybrid cells with the mutation for myoclonus epilepsy with ragged-red fibres ('MERRF') at position 8344 nt.

Authors:  H Antonická; D Floryk; P Klement; L Stratilová; J Hermanská; H Houstková; M Kalous; Z Drahota; J Zeman; J Houstek
Journal:  Biochem J       Date:  1999-09-15       Impact factor: 3.857

5.  Mutational spectrum of D-bifunctional protein deficiency and structure-based genotype-phenotype analysis.

Authors:  Sacha Ferdinandusse; Mari S Ylianttila; Jolein Gloerich; M Kristian Koski; Wendy Oostheim; Hans R Waterham; J Kalervo Hiltunen; Ronald J A Wanders; Tuomo Glumoff
Journal:  Am J Hum Genet       Date:  2005-11-15       Impact factor: 11.025

6.  A PEX6-defective peroxisomal biogenesis disorder with severe phenotype in an infant, versus mild phenotype resembling Usher syndrome in the affected parents.

Authors:  Annick Raas-Rothschild; Ronald J A Wanders; Petra A W Mooijer; Jeannette Gootjes; Hans R Waterham; Alisa Gutman; Yasuyuki Suzuki; Nobuyuki Shimozawa; Naomi Kondo; Gideon Eshel; Marc Espeel; Frank Roels; Stanley H Korman
Journal:  Am J Hum Genet       Date:  2002-02-28       Impact factor: 11.025

7.  Impaired degradation of phytanic acid in cells from patients with mitochondriopathies: evidence for the involvement of ETF and the respiratory chain in phytanic acid alpha-oxidation.

Authors:  R Fingerhut; W Schmitz; B Garavaglia; H Reichmann; E Conzelmann
Journal:  J Inherit Metab Dis       Date:  1994       Impact factor: 4.982

8.  Identification of PEX7 as the second gene involved in Refsum disease.

Authors:  Daan M van den Brink; Pedro Brites; Janet Haasjes; Anthony S Wierzbicki; John Mitchell; Michelle Lambert-Hamill; Jacqueline de Belleroche; Gerbert A Jansen; Hans R Waterham; Ronald J A Wanders
Journal:  Am J Hum Genet       Date:  2003-01-09       Impact factor: 11.025

9.  Comparison of fatty acid alpha-oxidation by rat hepatocytes and by liver microsomes fortified with NADPH, Fe3+ and phosphate.

Authors:  S Huang; P P Van Veldhoven; S Asselberghs; H J Eyssen; E de Hoffmann; G P Mannaerts
Journal:  Lipids       Date:  1994-10       Impact factor: 1.880

10.  Heimler Syndrome Is Caused by Hypomorphic Mutations in the Peroxisome-Biogenesis Genes PEX1 and PEX6.

Authors:  Ilham Ratbi; Kim D Falkenberg; Manou Sommen; Nada Al-Sheqaih; Soukaina Guaoua; Geert Vandeweyer; Jill E Urquhart; Kate E Chandler; Simon G Williams; Neil A Roberts; Mustapha El Alloussi; Graeme C Black; Sacha Ferdinandusse; Hind Ramdi; Audrey Heimler; Alan Fryer; Sally-Ann Lynch; Nicola Cooper; Kai Ren Ong; Claire E L Smith; Christopher F Inglehearn; Alan J Mighell; Claire Elcock; James A Poulter; Marc Tischkowitz; Sally J Davies; Abdelaziz Sefiani; Aleksandr A Mironov; William G Newman; Hans R Waterham; Guy Van Camp
Journal:  Am J Hum Genet       Date:  2015-09-17       Impact factor: 11.025
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