Biochemical Analysis in Peroxisomal Disorders View Full Text


Ontology type: schema:Chapter     


Chapter Info

DATE

1987

AUTHORS

R. J. A. Wanders , R. B. H. Schutgens , H. S. A. Heymans , J. Collins , S. Goldfischer , T. Hashimoto , G. Schrakamp , H. Van Den Bosch , J. M. Tager , A. W. Schram

ABSTRACT

Using immunoblotting analysis and in vitro peroxisomal β-oxidation activity measurements we show that in pseudo—Zellweger syndrome there is a deficiency of peroxisomal 2-oxoacyl-CoA thiolase. Finally, attention will be paid to the defect in X-linked adrenoleukodystrophy. In X-linked adrenoleukodystrophy there is an impairment in the peroxisomal oxidation of lignocerate but not of palmitate. Evidence will be presented that the two fatty acids are activated by different acyl-CoA ligases. Total lignoceroyl-CoA synthetase activity was, however, not deficient in X-linked adrenoleukodystrophy fibroblasts. More... »

PAGES

341-352

References to SciGraph publications

Book

TITLE

Peroxisomes in Biology and Medicine

ISBN

978-3-642-71327-9
978-3-642-71325-5

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/978-3-642-71325-5_36

DOI

http://dx.doi.org/10.1007/978-3-642-71325-5_36

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1024666749


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