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 journal › Article

80 Citations (Scopus)

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

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Adrenoleukodystrophy
Fibroblasts
Skin
Lipids
Fatty Acids
Sphingomyelins
Cell culture
Acids
Chemical analysis

ASJC Scopus subject areas

  • Biochemistry
  • Biophysics
  • Molecular Biology

Cite this

High concentration of hexacosanoate in cultured skin fibroblast lipids from adrenoleukodystrophy patients. / Kawamura, Nariko; Moser, Ann B.; Moser, Hugo W.; Ogino, Tadashi; Suzuki, Kunihiko; Schaumburg, Herbert; Milunsky, Aubrey; Murphy, Jerome; Kishimoto, Yasuo.

In: Biochemical and Biophysical Research Communications, Vol. 82, No. 1, 15.05.1978, p. 114-120.

Research output: Contribution to journal › Article

Kawamura, N, Moser, AB, Moser, HW, 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, vol. 82, no. 1, pp. 114-120. https://doi.org/10.1016/0006-291X(78)90584-3
Kawamura N, Moser AB, Moser HW, Ogino T, Suzuki K, Schaumburg H et al. High concentration of hexacosanoate in cultured skin fibroblast lipids from adrenoleukodystrophy patients. Biochemical and Biophysical Research Communications. 1978 May 15;82(1):114-120. https://doi.org/10.1016/0006-291X(78)90584-3
Kawamura, Nariko ; Moser, Ann B. ; Moser, Hugo W. ; Ogino, Tadashi ; Suzuki, Kunihiko ; Schaumburg, Herbert ; Milunsky, Aubrey ; Murphy, Jerome ; Kishimoto, Yasuo. / High concentration of hexacosanoate in cultured skin fibroblast lipids from adrenoleukodystrophy patients. In: Biochemical and Biophysical Research Communications. 1978 ; Vol. 82, No. 1. pp. 114-120.
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