X-linked adrenoleukodystrophy: Identification of the primary defect at the level of a deficient peroxisomal very long chain fatty acyl-CoA synthetase using a newly developed method for the isolation of peroxisomes from skin fibroblasts-Reference-Cited by-同舟云学术

X-linked adrenoleukodystrophy: Identification of the primary defect at the level of a deficient peroxisomal very long chain fatty acyl-CoA synthetase using a newly developed method for the isolation of peroxisomes from skin fibroblasts

Published:1988-06 Issue:S2 Volume:11 Page:173-177
ISSN:0141-8955
Container-title:Journal of Inherited Metabolic Disease
language:en
Short-container-title:J Inherit Metab Dis

Author:

Wanders R. J. A.,van Roermund C. W. T.,van Wijland M. J. A.,Schutgens R. B. H.,Schram A. W.,Tager J. M.,van den Bosch H.,Schalkwijk C.

Publisher

Wiley

Subject

Genetics(clinical),Genetics

Link

http://link.springer.com/content/pdf/10.1007/BF01804228

Reference10 articles.

1. Hashmi, M., Stanley, W. and Singh, I. Lignoceroyl-CoASH ligase: enzyme defect in fatty acidβ-oxidation in X-linked adrenoleukodystrophy.FEBS Lett. 86 (1986) 247–250

2. Moser, H. W., Moser, A. B., Singh, I. and O'Neill, B. P. Adrenoleukodystrophy: survey of 303 cases: biochemistry, diagnosis and therapy.Ann. Neurol. 16 (1984) 628–641

3. Rizzo, W. B., Avigan, J., Chemke, J. and Schulman, J. D. Adrenoleukodystrophy: very long chain fatty acid metabolism in fibroblasts.Neurology 34 (1984) 163–169

4. Singh, I., Moser, A. E., Goldfischer, S. and Moser, H. W. Lignoceric acid is oxidized in the peroxisome: implications for the Zellweger cerebro-hepato-renal syndrome and adrenoleukodystrophy.Proc. Natl. Acad. Sci. USA 81 (1984) 4203–4207

5. Singh, I., Moser, H. W., Moser, A. E. and Kishimoto, Y. Adrenoleukodystrophy: impaired oxidation of long chain fatty acids in cultured skin fibroblasts and adrenal cortex.Biochem. Biophys. Res. Commun. 102 (1981) 1223–1229

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