Download PDF
Neuropediatrics 1990; 21(3): 119-123
DOI: 10.1055/s-2008-1071476
Original article

© Georg Thieme Verlag KG Stuttgart · New York

Analysis of Very Long-Chain Fatty Acids and Plasmalogen in the Erythrocyte Membrane: A Simple Method for the Detection of Peroxisomal Disorders and Discrimination Between Adrenoleukodystrophy and Zellweger Syndrome

K.  Tanaka1 , K.  Nishizawa2 , H.  Yamamoto2 , T.  Naruto2 , E.  Izeki2 , T.  Taga2 , M.  Shimada2 , Y.  Saeki3
  • 1Department of Pediatrics, Nagahama Red Cross Hospital, Nagahama, Japan
  • 2Department of Pediatrics, Shiga University of Medical Science, Otsu, Japan
  • 3Department of Central Research Laboratory, Shiga University of Medical Science, Otsu, Japan
Further Information

Publication History

Publication Date:
19 March 2008 (online)

  PDF Download  Permissions and Reprints

Abstract

We analyzed the sphingomyelin very long-chain fatty acids (VLCFAs) and phosphatidylethanolamine (PE) plasmalogen contents of the erythrocyte membrane in patients suffering from peroxisomal disorders. In a patient with Zellweger syndrome, both a decrease in the PE plasmalogen content and an increase in the sphingomyelin VLCFAs content of the erythrocyte membrane were noted. In patients with adrenoleukodystrophy, however, there was no decrease in PE plasmalogen, although the sphingomyelin VLCFAs content of the membrane was significantly increased in comparison with control values. Analyses of both sphingomyelin VLCFAs and PE plasmalogen were carried out simultaneously, using both the same process and the same sample.

Key words

Peroxisomes - Very long-chain fatty acids - Phosphatidylethanolamine plasmalogen - Zellweger syndrome - Adrenoleukodystrophy

      >