Immunochemical and biochemical studies of fatty acid oxidation in fibroblasts of Zellweger and X-linked adrenoleukodystrophy patients
| Autor: | F.A.G. Reubsaet, J.H. Veerkamp, L.A.H. Monnens, J. M. F. Trijbels, Takashi Hashimoto, M.L.P. Brückwilder |
|---|---|
| Rok vydání: | 1991 |
| Předmět: |
X Chromosome
Immunoblotting Biophysics Palmitic Acid Lignoceric acid Palmitic Acids Biology Biochemistry Cerebrohepatorenal syndrome Microbodies chemistry.chemical_compound Endocrinology Peroxisomal disorder medicine Humans Adrenoleukodystrophy Zellweger Syndrome Beta oxidation Zellweger syndrome Thiolase Fatty Acids Peroxisome Fibroblasts medicine.disease Acetyl-CoA C-Acyltransferase chemistry Acyl-CoA Oxidase Oxidoreductases Oxidation-Reduction |
| Zdroj: | Biochimica et biophysica acta. 1083(3) |
| ISSN: | 0006-3002 |
| Popis: | Immunoblot analyses of peroxisomal β-oxidation enzymes showed that subunit A of acyl-CoA oxidase gave a stronger immunoreaction in fibroblasts of Zellweger and X-linked adrenoleukodystrophy patients than in those of controls. Subunits B and C and 3-ketoacyl-CoA thiolase were detected in fibroblasts of controls and X-linked adrenoleukodystrophy patients, but not of Zellweger patients. Total oxidation of palmitic and lignoceric acid was normal in homogenates of fibroblasts from Zellweger and X-linked adrenoleukodystrophy patients. The peroxisomal oxidation of both acids was only deficient in Zellweger patients. These data may not reflect the situation in vivo, as is evident from the accumulation of very-long-chain fatty acids in Zellweger and X-linked adrenoleukodystrophy patients. |
| Databáze: | OpenAIRE |
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