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J Greter, S Lindstedt, G Steen, 2,6-Dimethyloctanedioic acid--a metabolite of phytanic acid in Refsum's disease., Clinical Chemistry, Volume 29, Issue 3, 1 March 1983, Pages 434–437, https://doi.org/10.1093/clinchem/29.3.434
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Abstract
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.
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© 1983 The American Association of Clinical Chemists, Inc.
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