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Adrenoleukodystrophy: abnormality of very long-chain fatty acids in erythrocyte membrane phospholipids.

作者信息

Antoku Y, Sakai T, Goto I, Iwashita H, Kuroiwa Y

出版信息

Neurology. 1984 Nov;34(11):1499-1501. doi: 10.1212/wnl.34.11.1499.

DOI:10.1212/wnl.34.11.1499
PMID:6493501
Abstract

Long-chain fatty acids in erythrocyte membrane phospholipids were analyzed in three patients with adrenoleukodystrophy (ALD) and four diseased controls with other neurologic diseases by high performance liquid chromatography (HPLC). HPLC chromatograms showed the increase of a very long-chain fatty acid C26:0 in glycerophospholipids of ALD erythrocyte membranes, which was not so prominent in sphingomyelin. The ratio of C26:0 to C22:0 increased in glycerophospholipids as well as sphingomyelin in ALD. These results suggest that ALD has a generalized abnormal metabolism of very long-chain saturated fatty acids.

摘要

相似文献

1
Adrenoleukodystrophy: abnormality of very long-chain fatty acids in erythrocyte membrane phospholipids.
Neurology. 1984 Nov;34(11):1499-1501. doi: 10.1212/wnl.34.11.1499.
2
Very long-chain fatty acids in erythrocyte membrane sphingomyelin: detection of ALD hemizygotes and heterozygotes.红细胞膜鞘磷脂中的极长链脂肪酸:肾上腺脑白质营养不良半合子和杂合子的检测。
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Adrenoleukodystrophy: fatty acid analysis of total glycerophospholipids in erythrocyte membranes.
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Very long-chain fatty acids in neutral lipids and glycerophospholipids of adrenoleukodystrophy-cultured skin fibroblasts.
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Plasmalogen analysis in erythrocyte membranes of adrenoleukodystrophy.
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Adrenoleukodystrophy: detection of increased very long chain fatty acids by high-performance liquid chromatography.肾上腺脑白质营养不良:通过高效液相色谱法检测极长链脂肪酸增加
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Very long-chain fatty acids in erythrocyte membrane phospholipids in adrenoleukodystrophy.肾上腺脑白质营养不良患者红细胞膜磷脂中的极长链脂肪酸
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Adrenoleukodystrophy: impaired oxidation of very long chain fatty acids in white blood cells, cultured skin fibroblasts, and amniocytes.肾上腺脑白质营养不良:白细胞、培养的皮肤成纤维细胞和羊膜细胞中极长链脂肪酸氧化受损。
Pediatr Res. 1984 Mar;18(3):286-90. doi: 10.1203/00006450-198403000-00016.

引用本文的文献

1
Very long chain fatty acids in higher animals--a review.高等动物中的极长链脂肪酸——综述
Lipids. 1995 Jan;30(1):1-14. doi: 10.1007/BF02537036.
2
Adrenoleukodystrophy without adrenal insufficiency and its magnetic resonance imaging.无肾上腺功能不全的肾上腺脑白质营养不良及其磁共振成像
J Neurol. 1985;232(5):265-70. doi: 10.1007/BF00313862.
3
Very-long-chain fatty acids from lower organism.来自低等生物的极长链脂肪酸。
Folia Microbiol (Praha). 1987;32(2):149-76. doi: 10.1007/BF02883244.
4
MR imaging of adrenoleukodystrophy.肾上腺脑白质营养不良的磁共振成像
Neuroradiology. 1991;33(1):25-9. doi: 10.1007/BF00593329.