Method for Measurement of Peroxisomal Very-Long-Chain Fatty Acid β-Oxidation in Human Skin Fibroblasts Using Stable-Isotope-Labeled Tetracosanoic Acid-Reference-Cited by-同舟云学术

Method for Measurement of Peroxisomal Very-Long-Chain Fatty Acid β-Oxidation in Human Skin Fibroblasts Using Stable-Isotope-Labeled Tetracosanoic Acid

Published:2004-10-01 Issue:10 Volume:50 Page:1824-1826
ISSN:0009-9147
Container-title:Clinical Chemistry
language:en
Short-container-title:

Author:

Kemp Stephan¹,Valianpour Fredoen¹,Mooyer Petra A W¹,Kulik Willem¹,Wanders Ronald J A¹

Affiliation:

1. University of Amsterdam, Academic Medical Center, Departments of Pediatrics/Emma Children’s Hospital, and Clinical Chemistry, Laboratory for Genetic Metabolic Diseases, Amsterdam, The Netherlands

Publisher

Oxford University Press (OUP)

Subject

Biochemistry, medical,Clinical Biochemistry

Link

http://academic.oup.com/clinchem/article-pdf/50/10/1824/32737215/clinchem1824.pdf

Reference8 articles.

1. Lignoceric acid is oxidized in the peroxisome: implications for the Zellweger cerebro-hepato-renal syndrome and adrenoleukodystrophy.

2. Adrenoleukodystrophy: Impaired Oxidation of Very Long Chain Fatty Acids in White Blood Cells, Cultured Skin Fibroblasts, and Amniocytes

3. Peroxisomal lignoceroyl-CoA ligase deficiency in childhood adrenoleukodystrophy and adrenomyeloneuropathy.

4. Direct demonstration that the deficient oxidation of very long chain fatty acids in X-linked adrenoleukodystrophy is due to an impaired ability of peroxisomes to activate very long chain fatty acids

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