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 | |
| State | Published - May 15 1978 |
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ASJC Scopus subject areas
- Biochemistry
- Biophysics
- Molecular Biology
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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
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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
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.
UR - http://www.scopus.com/inward/record.url?scp=0017823289&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0017823289&partnerID=8YFLogxK
U2 - 10.1016/0006-291X(78)90584-3
DO - 10.1016/0006-291X(78)90584-3
M3 - Article
C2 - 666828
AN - SCOPUS:0017823289
VL - 82
SP - 114
EP - 120
JO - Biochemical and Biophysical Research Communications
JF - Biochemical and Biophysical Research Communications
SN - 0006-291X
IS - 1
ER -