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Science 4 January 1985:
Vol. 227. no. 4682, pp. 67 - 70
DOI: 10.1126/science.3964959
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Articles

Science, Vol 227, Issue 4682, 67-70
Copyright © 1985 by American Association for the Advancement of Science

articles

Peroxisomal defects in neonatal-onset and X-linked adrenoleukodystrophies

S Goldfischer, J Collins, I Rapin, B Coltoff-Schiller, CH Chang, M Nigro, VH Black, NB Javitt, HW Moser, and PB Lazarow

Accumulation of very long chain fatty acids in X-linked and neonatal forms of adrenoleukodystrophy (ALD) appears to be a consequence of deficient peroxisomal oxidation of very long chain fatty acids. Peroxisomes were readily identified in liver biopsies taken from a patient having the X-linked disorder. However, in liver biopsies from a patient having neonatal-onset ALD, hepatocellular peroxisomes were greatly reduced in size and number, and sedimentable catalase was markedly diminished. The presence of increased concentrations of serum pipecolic acid and the bile acid intermediate, trihydroxycoprostanic acid, in the neonatal ALD patient are associated with a generalized diminution of peroxisomal activities that was not observed in the patient with X-linked ALD.


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
Peroxisomal Proliferation Protects from {beta}-Amyloid Neurodegeneration.
M. J. Santos, R. A. Quintanilla, A. Toro, R. Grandy, M. C. Dinamarca, J. A. Godoy, and N. C. Inestrosa (2005)
J. Biol. Chem. 280, 41057-41068
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Mitochondrial Alterations Caused by Defective Peroxisomal Biogenesis in a Mouse Model for Zellweger Syndrome (PEX5 Knockout Mouse).
E. Baumgart, I. Vanhorebeek, M. Grabenbauer, M. Borgers, P. E. Declercq, H. D. Fahimi, and M. Baes (2001)
Am. J. Pathol. 159, 1477-1494
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Two Siblings with Phenotypes Mimicking Peroxisomal Disorders but with Discordant Biochemical Findings.
J. J. Pietrzyk, D. Turowska-Heydel, M. Klimek, F. Kaczmarski, and J. Kaluza (1990)
Clinical Pediatrics 29, 479-484
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Linkage of DNA Markers at Xq28 to Adrenoleukodystrophy and Adrenomyeloneuropathy Present Within the Same Family.
P. J. Willems, L. Vits, R. J. A. Wanders, P. J. Coucke, B. J. Van der Auwera, A. F. Van Elsen, P. Raeymaekers, C. Van Broeckhoven, R. B. H. Schutgens, G. Dacremont, et al. (1990)
Arch Neurol 47, 665-669
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Review Article: The Inherited Neurodegenerative Disorders of Childhood: Clinical Assessment.
A. K. Percy (1987)
J Child Neurol 2, 82-97
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Adrenoleukodystrophy: From Bedside to Molecular Biology.
H. W. Moser (1987)
J Child Neurol 2, 140-150
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