PHYTANIC ACID FORMATION AND ACCUMULATION IN PHYTOL-FED RATS.

PHYTANIC ACID FORMATION AND ACCUMULATION IN PHYTOL-FED RATS.
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植醇喂养大鼠中植酸的形成和积累。

DOI:
10.1016/0006-291x(65)90138-5
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发表时间:
1965
影响因子:
3.1
通讯作者:
J. Baxter
J. Baxter
中科院分区:
生物学4区
文献类型:
--
作者:
D. Steinberg;J. Avigan;C. Mize;J. Baxter

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Klenk和Kahlke(1963)和Richterich等(1963)报道了遗传性失调性多发性神经炎(Refsum‘s病)患者的血液和死后组织中存在高浓度的植酸(3,7,11,15-四甲基肉豆蔻酸)。这种疾病最初是由Refsum(1946)描述为临床实体,他认识到它是遗传的、隐性的,并推测可归因于特定的代谢错误(Refsum,1960)。主要临床特征包括周围肥厚性神经病、共济失调和视网膜色素变性。现在已经在至少9例Refsum病患者的血浆中证明了植酸(Kahlke,1964),但这种酸的来源仍有待确定,这种酸在正常人的血浆中尚未报道。在黄油脂肪(Hansen and Ghorland,1953;Sonneveld et al,1962)、牛血浆(Lough,1964)和牛肾周脂肪(Hansen,1965)中都发现了植酸,但浓度很低。植醇(3,7,11,-15-四甲基十六碳-2-烯-1-醇)是叶绿素分子的主要成分(占其质量的L/3),如果被吸收并转化为植酸,可能是一种重要的饮食来源。通过气-液色谱(GLC)和核磁共振波谱分析,验证了其纯度(>905)。以植醇为原料,分三步合成植酸:用二氧化锰氧化成醛;将醛氧化成邻苯二甲酸(3,7,11,15-四甲基十六碳-2-环酸)。
The presence of high concentrations of phytanic acid (3, 7, 11, 15-tetramethylmyristic acid) in the lipids of blood and post-mortem tissues of a patient with heredopathia atactica polyneuritiformis(Refsum's disease) has been reported by Klenk and Kahlke (1963) and by Richterich et al (1963). This disease was first described as a clinical entity by Refsum (1946) who recognized it as genetically transmitted, recessive, and presumably attributable to a specific metabolic error (Refsum, 1960). The primary clinical features include peripheral hypertrophic neuropathy, ataxia and retinitis pigmentosa. Phytanic acid has now been demonstrated in the plasma lipids of at least nine cases of Refsum's disease (Kahlke, 1964), but the origin of this acid, which has not been reported in the plasma of normal individuals, remains to be established. Phytanic acid has been found in butter fat (Hansen and Ghorland, 1953; Sonneveld et al, 1962), ox plasma (Lough, l964), and ox perinephric fat (Hansen, l965), but the concentrations are very low. Phytol (3, 7, 11,-15-tetramethylhexadec-2-en-1-ol), which is a major constituent of the chlorophyll molecule (accounting for l/3 of its mass), might represent a significant dietary source, if absorbed and converted to phytanic acid. Phytol was obtained commercially and its purity (> 905) was verified by gas-liquid chromatography (GLC) and by analysis of its nuclear magnetic resonance spectrum. Phytanic acid was synthesized from phytol in three steps: oxidation to the aldehyde with manganese dioxide; oxidation of the aldehyde to PhYtenic acid (3, 7, 11, 15-tetramethylhexadec-2-epoic acid)