Peroxisomal disorders: Complementation analysis using beta-oxidation of very long chain fatty acids

M. C. McGuinness, A. B. Moser, H. W. Moser, P. A. Watkins

Research output: Contribution to journalArticle

Abstract

Complementation studies, using fused cell lines from patients with peroxisomal disorders, have shown correction of defective plasmalogen synthesis and phytanic acid oxidation as wll as an increase in the number of peroxisomes. At least six complementation groups have been reported. We demostrate here that complementing cell lines also acquire the ability to oxidize very long chain fatty acids (VLCFA), and that complementation groups defined with this technique are identical to those reported previously when plasmalogen synthesis was used as the criterion for complementation. This VLCFA complementation technique is of particular value in the study of patients in whom defective VLCFA is the only or major enzymatic defect, and we show complementation between cell lines from two patients each with an isolated defect in one of the peroxisomal fatty acid beta-oxidation enzymes.

Original languageEnglish (US)
Pages (from-to)364-369
Number of pages6
JournalBiochemical and Biophysical Research Communications
Volume172
Issue number1
DOIs
StatePublished - Oct 15 1990

ASJC Scopus subject areas

  • Biophysics
  • Biochemistry
  • Molecular Biology
  • Cell Biology

Fingerprint Dive into the research topics of 'Peroxisomal disorders: Complementation analysis using beta-oxidation of very long chain fatty acids'. Together they form a unique fingerprint.

  • Cite this