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
Kishimoto,Y
中科院分区:
生物学4区
文献类型:
--
作者:
Singh,I;Moser,HW;Moser,AB;Kishimoto,Y

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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: --
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影响因子: 4.8
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期刊: The Journal of biological chemistry
影响因子: --
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