Adrenoleukodystrophy: impaired oxidation of long chain fatty acids in cultured skin fibroblasts an adrenal cortex.
Adrenoleukodystrophy: impaired oxidation of long chain fatty acids in cultured skin fibroblasts an adrenal cortex.
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肾上腺脑白质营养不良:培养的皮肤成纤维细胞和肾上腺皮质中长链脂肪酸的氧化受损。
DOI:
10.1016/s0006-291x(81)80142-8
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发表时间:
1981
影响因子:
3.1
通讯作者:
Kishimoto,Y
中科院分区:
文献类型:
--
作者:
Singh,I;Moser,HW;Moser,AB;Kishimoto,Y
CO2production from [1-14C] fatty acids was studied with cultured skin fibroblasts and adrenal gland from adrenoleukodystrophy (ALD) patients. The CO2production from lignoceric acid in 11 and from hexacosanoic acid in 4 ALD cultured skin fibroblasts cultures was reduced to 8.2 ± 9 and 14 ± 8% of control, respectively. The adrenal studies compared a biopsy specimen from a 7 year old boy with ALD and 6 control adrenals from renal transplant donors. CO2production from oleic acid was reduced to less than 10% of control, while cholesterol esterase and cholesterol esterifying enzyme activities were similar to the control.
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DOI:
10.1016/s0021-9258(19)63332-6
发表时间:
1977
期刊:
The Journal of biological chemistry
影响因子:
--
作者:
S. Murad;R. Chen;Y. Kishimoto
通讯作者:
Y. Kishimoto
影响因子:
4.7
作者:
M. Igarashi;H. Schaumburg;J. Powers;Y. Kishimoto;E. Koilodny;K. Suzuki
通讯作者:
K. Suzuki
影响因子:
3.6
作者:
V. Michels;Arthur L Beaudet
通讯作者:
Arthur L Beaudet
影响因子:
4.8
作者:
M. Hoshi;Y. Kishimoto
通讯作者:
Y. Kishimoto
DOI:
10.1016/s0021-9258(19)41129-0
发表时间:
1975
期刊:
The Journal of biological chemistry
影响因子:
--
作者:
S. Murad;Y. Kishimoto
通讯作者:
Y. Kishimoto