Clinical Chemistry 1983-03-01

2,6-Dimethyloctanedioic acid--a metabolite of phytanic acid in Refsum's disease.

J Greter, S Lindstedt, G Steen

文献索引：Clin. Chem. 29(3) , 434-7, (1983)

全文：HTML全文

摘要

The urine of two patients with Refsum's disease consistently contained 2,6-dimethyloctanedioic acid, a compound not normally found in human urine. In addition, their urines contained increased concentrations of 3-methylhexanedioic acid. These two compounds may be formed from phytanic acid by an initial omega-oxidation and subsequent beta-oxidations. It was calculated that this oxidation pathway may metabolize at least 30 mg of phytanic acid per day.

相关化合物

  • 六氢吡啶-alpha-羧...
  • 甲基丁二酸
  • 植烷酸
  • 3-甲基己二酸

相关文献：

Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression.

2009-02-12

[Nature 457(7231) , 910-4, (2009)]

Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease.

[J. Chromatogr. A. 562(1-2) , 125-38, (1991)]

ECNI GC-MS analysis of picolinic and quinolinic acids and their amides in human plasma, CSF, and brain tissue.

2003-01-01

[Adv. Exp. Med. Biol. 527 , 705-12, (2003)]

Neuron-specific enolase, nucleotides, nucleosides, purine bases, oxypurines and uric acid concentrations in cerebrospinal fluid of children with meningitis.

2003-03-01

[Brain Dev. 25(2) , 102-6, (2003)]

Cerebrospinal fluid taurine after traumatic brain injury.

2005-01-01

[Neurochem. Res. 30(1) , 123-8, (2005)]

更多文献...