High concentration of hexacosanoate in cultured skin fibroblast lipids from adrenoleukodystrophy patients

Nariko Kawamura, Ann B. Moser, Hugo W. Moser, Tadashi Ogino, Kunihiko Suzuki, Herbert Schaumburg, Aubrey Milunsky, Jerome Murphy, Yasuo Kishimoto

Research output: Contribution to journalArticle

80 Scopus citations

Abstract

Cultured skin fibroblasts from nine patients with the clinical diagnosis of adrenoleukodystrophy (ALD) and 16 control individuals were studied for fatty acid compositions. Total lipids from 7 of the 9 ALD samples showed a high content of hexacosanoic acid (26:0) and a higher ratio of 26:0 to 22:0 acids (0.78-0.92) than the controls (0.03 to 0.09). The samples obtained from two patients with atypical marifestations had a ratio similar to control values. A similar abnormality was demonstrated in the sphingomyelin fatty acids. Diagnosis of ALD and study of its disease mechanism may thus be feasible in skin fibroblast cultures.

Original languageEnglish (US)
Pages (from-to)114-120
Number of pages7
JournalBiochemical and Biophysical Research Communications
Volume82
Issue number1
DOIs
StatePublished - May 15 1978

ASJC Scopus subject areas

  • Biophysics
  • Biochemistry
  • Molecular Biology
  • Cell Biology

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    Kawamura, N., Moser, A. B., Moser, H. W., Ogino, T., Suzuki, K., Schaumburg, H., Milunsky, A., Murphy, J., & Kishimoto, Y. (1978). High concentration of hexacosanoate in cultured skin fibroblast lipids from adrenoleukodystrophy patients. Biochemical and Biophysical Research Communications, 82(1), 114-120. https://doi.org/10.1016/0006-291X(78)90584-3