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Measurement of peroxisomal fatty acid beta-oxidation in cultured human skin fibroblasts.
- Source :
-
Journal of inherited metabolic disease [J Inherit Metab Dis] 1995; Vol. 18 Suppl 1, pp. 113-24. - Publication Year :
- 1995
-
Abstract
- One of the main functions of mammalian peroxisomes is the beta-oxidation of a variety of fatty acids and fatty acid derivatives, including very long-chain fatty acids. Oxidation of these fatty acids is deficient in a number of different peroxisomal disorders, including the disorders of peroxisome biogenesis (Zellweger syndrome, neonatal adrenoleukodystrophy and infantile Refsum disease), X-linked adrenoleukodystrophy and a number of other disorders of peroxisomal beta-oxidation of known and unknown aetiology. Accurate measurement of peroxisomal fatty acid oxidation is of utmost importance for correct postnatal and prenatal diagnosis of these disorders. In this paper we describe a straightforward and accurate assay method to measure the beta-oxidation of palmitic acid (C16:0), hexacosanoic acid (C26:0) and pristanic acid in intact fibroblasts.
- Subjects :
- Cell Adhesion
Cells, Cultured
Fatty Acids metabolism
Female
Fibroblasts
Humans
Indicators and Reagents
Oxidation-Reduction
Palmitic Acids metabolism
Peroxisomal Disorders metabolism
Pregnancy
Prenatal Diagnosis
Proteins analysis
Proteins metabolism
Skin cytology
Fatty Acids chemistry
Microbodies chemistry
Peroxisomal Disorders diagnosis
Skin metabolism
Subjects
Details
- Language :
- English
- ISSN :
- 0141-8955
- Volume :
- 18 Suppl 1
- Database :
- MEDLINE
- Journal :
- Journal of inherited metabolic disease
- Publication Type :
- Academic Journal
- Accession number :
- 9053546
- Full Text :
- https://doi.org/10.1007/BF00711434