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The occurrence of substituted 3-methyl-3-hydroxyglutaric acids in urine in propionic acidaemia and in β-ketothiolase deficiency (1983)

Pollitt, R. J.

Chichester : Wiley-Blackwell

Biological Mass Spectrometry 10 (1983), S. 253-257

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ISSN: 0306-042X

Keywords: Chemistry ; Analytical Chemistry and Spectroscopy

Source: Wiley InterScience Backfile Collection 1832-2000

Topics: Chemistry and Pharmacology

Notes: The urine of two patients with propionic acidaemia contained 3-ethyl-3-hydroxyglutaric acid which has a propionyl residue in place of one of the acetyl residues of the normal metabolite 3-methyl-3-hydroxyglutaric acid. Related compounds, 2,3-dimethyl-3-hydroxygIutaric acid, 2-methyl-3-ethyl-3-hydroxyglutaric acid and 2,3,4-trimethyl-3-hydroxyglutaric acid, could not be detected in propionic acidaemia urine, but 2,3-dimethyl-3-hydroxyglutaric acid was excreted by a patient with β-ketothiolase deficiency.

Additional Material: 4 Ill.

Type of Medium: Electronic Resource

URL: http://dx.doi.org/10.1002/bms.1200100403

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