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Occurrence of novel branched-chain fatty acids in Refsum's disease.

作者信息

Dulaney J T, Williams M, Evans J E, Costello C E, Kolodny E H

出版信息

Biochim Biophys Acta. 1978 Apr 28;529(1):1-12. doi: 10.1016/0005-2760(78)90097-8.

DOI:10.1016/0005-2760(78)90097-8
PMID:76480
Abstract

Two novel branched-chain fatty acids, which appear to be unsaturated analogs of phytanic acid, have been observed in sera and urine of patients with Refsum's disease. They occur in both phospholipids and neutral lipids, and have been isolated and characterized.

摘要

相似文献

1
Occurrence of novel branched-chain fatty acids in Refsum's disease.
Biochim Biophys Acta. 1978 Apr 28;529(1):1-12. doi: 10.1016/0005-2760(78)90097-8.
2
Metabolism of phytanic acid in Refsum's disease.
Lancet. 1982 Jan 23;1(8265):194-6. doi: 10.1016/s0140-6736(82)90760-7.
3
2,6-Dimethyloctanedioic acid--a metabolite of phytanic acid in Refsum's disease.
Clin Chem. 1983 Mar;29(3):434-7.
4
Fatty acid composition of tissues in Refsum's disease (herodopathia atactica polyneuritiformis). Estimation of total phytanic acid accumulation.雷夫叙姆病(多神经炎型遗传性共济失调)组织中的脂肪酸组成。植烷酸总蓄积量的评估。
Biomedicine. 1974 Nov;20(6):398-407.
5
Refsum's disease and metabolism of phytanic acid.雷夫叙姆病与植烷酸代谢
Lancet. 1982 Apr 17;1(8277):906-7. doi: 10.1016/s0140-6736(82)92172-9.
6
Tissue distribution of phytanic acid and its analogues in a kinship with Refsum's disease.植烷酸及其类似物在与雷夫叙姆病相关的亲属关系中的组织分布。
Lipids. 1987 Feb;22(2):69-75. doi: 10.1007/BF02534855.
7
Infantile Refsum's disease (phytanic acid storage disease): a variant of Zellweger's syndrome?婴儿型雷夫叙姆病(植烷酸贮积病):泽尔韦格综合征的一种变异型?
Clin Genet. 1984 Dec;26(6):579-86. doi: 10.1111/j.1399-0004.1984.tb01107.x.
8
Phytanic acid alpha-oxidation and complementation analysis of classical Refsum and peroxisomal disorders.植烷酸α-氧化及经典型雷夫叙姆病和过氧化物酶体疾病的互补分析
Hum Genet. 1989 Jan;81(2):175-81. doi: 10.1007/BF00293897.
9
Phytanic acid and very long chain fatty acids in genetic peroxisomal disorders.遗传性过氧化物酶体疾病中的植烷酸和极长链脂肪酸。
J Clin Chem Clin Biochem. 1989 May;27(5):309-14.
10
Disorders related to the metabolism of phytanic acid.与植烷酸代谢相关的疾病。
Scand J Clin Lab Invest Suppl. 1986;184:3-10.

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4
Accumulation of pristanic acid (2, 6, 10, 14 tetramethylpentadecanoic acid) in the plasma of patients with generalised peroxisomal dysfunction.原发性过氧化物酶体功能障碍患者血浆中降植烷酸(2,6,10,14-四甲基十五烷酸)的蓄积。
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5
Tissue distribution of phytanic acid and its analogues in a kinship with Refsum's disease.植烷酸及其类似物在与雷夫叙姆病相关的亲属关系中的组织分布。
Lipids. 1987 Feb;22(2):69-75. doi: 10.1007/BF02534855.