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

doi:10.1016/0006-291X(78)90584-3

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

Neurology

Research output: Contribution to journal › Article › peer-review

84 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 language	English (US)
Pages (from-to)	114-120
Number of pages	7
Journal	Biochemical and Biophysical Research Communications
Volume	82
Issue number	1
DOIs	https://doi.org/10.1016/0006-291X(78)90584-3
State	Published - May 15 1978

ASJC Scopus subject areas

Biophysics
Biochemistry
Molecular Biology
Cell Biology

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10.1016/0006-291X(78)90584-3

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@article{b1ae9772acfb4a86b97069c069c40efb,

title = "High concentration of hexacosanoate in cultured skin fibroblast lipids from adrenoleukodystrophy patients",

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.",

author = "Nariko Kawamura and Moser, \{Ann B.\} and Moser, \{Hugo W.\} and Tadashi Ogino and Kunihiko Suzuki and Herbert Schaumburg and Aubrey Milunsky and Jerome Murphy and Yasuo Kishimoto",

note = "Funding Information: ACKNOWLEDGEMENTS: The authors thank Ms. Catherine Rappe for her excellent technical assistance. This research was supported in part by NIH research grants HD10981, NS13559, NS13569, NS13513, NS10885, NS03356, HDD7799, HD05515 from the United States Public Health Service, National Foundation 6-142, and Maternal and Child Health Project 917.",

year = "1978",

month = may,

day = "15",

doi = "10.1016/0006-291X(78)90584-3",

language = "English (US)",

volume = "82",

pages = "114--120",

journal = "Biochemical and Biophysical Research Communications",

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publisher = "Elsevier B.V.",

number = "1",

}

TY - JOUR

T1 - High concentration of hexacosanoate in cultured skin fibroblast lipids from adrenoleukodystrophy patients

AU - Kawamura, Nariko

AU - Moser, Ann B.

AU - Moser, Hugo W.

AU - Ogino, Tadashi

AU - Suzuki, Kunihiko

AU - Schaumburg, Herbert

AU - Milunsky, Aubrey

AU - Murphy, Jerome

AU - Kishimoto, Yasuo

N1 - Funding Information: ACKNOWLEDGEMENTS: The authors thank Ms. Catherine Rappe for her excellent technical assistance. This research was supported in part by NIH research grants HD10981, NS13559, NS13569, NS13513, NS10885, NS03356, HDD7799, HD05515 from the United States Public Health Service, National Foundation 6-142, and Maternal and Child Health Project 917.

PY - 1978/5/15

Y1 - 1978/5/15

N2 - 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.

AB - 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.

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U2 - 10.1016/0006-291X(78)90584-3

DO - 10.1016/0006-291X(78)90584-3

M3 - Article

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AN - SCOPUS:0017823289

SN - 0006-291X

VL - 82

SP - 114

EP - 120

JO - Biochemical and Biophysical Research Communications

JF - Biochemical and Biophysical Research Communications

IS - 1

ER -

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

Abstract

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