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  • Title: Urinary excretion of 2-methyl-2,3-butanediol and 2,3-pentanediol in patients with disorders of propionate and methylmalonate metabolism.
    Author: Duran M, Ketting D, Bruinvis L, Dorland L, Wadman SK, Bakkeren JA.
    Journal: Clin Chim Acta; 1987 Jun 15; 165(2-3):197-204. PubMed ID: 3652445.
    Abstract:
    Urine samples from patients with propionic acidemia and from a patient with methylmalonic acidemia contained unknown non-acidic metabolites by gas chromatography/mass spectrometry after ethyl acetate extraction. It could be demonstrated by mass spectrometric studies and by synthesis of reference compounds that the major metabolite was 2-methyl-2,3-butanediol, while smaller amounts of 2,3-pentanediol were also present. These diols were present in abnormal amounts in these patients during attacks of metabolic decompensation.
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