Very long-chain fatty acids in erythrocyte membrane sphingomyelin: detection of ALD hemizygotes and heterozygotes.

Abstract

We used mass fragmentography to analyze a very long-chain fatty acid composition of erythrocyte membrane sphingomyelin. The content of hexacosanoic acid (C26:0), tetracosanoic acid (C24:0) and docosanoic acid (C22:0) in adrenoleukodystrophy (ALD) hemizygotes and heterozygotes was significantly higher than in controls. Although the ratio of C26:0 to C22:0 in 18 of the 46 controls was in the range of ALD heterozygotes, the absolute content of C26:0 and C24:0 in controls did not overlap with ALD hemizygotes or heterozygotes. The content of C26:0 and C24:0 is more reliable for detection of ALD hemizygotes and heterozygotes than the ratio of C26:0 to C22:0 when red cell sphingomyelin is used for diagnosis.

• Authors: K Tanaka, M Shimada, T Naruto, H Yamamoto, Y Saeki, H Sai, G Hirose
• Journal: Neurology (Neurology) Vol. 36 Issue 6 Pg. 791-5 (Jun 1986) ISSN: 0028-3878 [Print] United States
• PMID: 3703284 (Publication Type: Journal Article)

Chemical References

• Fatty Acids
• Sphingomyelins

Topics

• Adolescent
• Adrenoleukodystrophy (genetics, metabolism)
• Adult
• Child
• Diffuse Cerebral Sclerosis of Schilder (metabolism)
• Erythrocyte Membrane (analysis)
• Fatty Acids (analysis, genetics)
• Female
• Heterozygote
• Humans
• Male
• Sphingomyelins (analysis, genetics)