Excessive excretion of β-alanine and of 3-hydroxypropionic,R- andS-3-aminoisobutyric,R- andS-3-hydroxyisobutyric andS-2-(hydroxymethyl)butyric acids probably due to a defect in the metabolism of the corresponding malonic semialdehydes

@article{Pollitt1985ExcessiveEO,
  title={Excessive excretion of β-alanine and of 3-hydroxypropionic,R- andS-3-aminoisobutyric,R- andS-3-hydroxyisobutyric andS-2-(hydroxymethyl)butyric acids probably due to a defect in the metabolism of the corresponding malonic semialdehydes},
  author={Rodney J. Pollitt and Anne Green and Rebecca K. Smith},
  journal={Journal of Inherited Metabolic Disease},
  year={1985},
  volume={8},
  pages={75-79}
}
A new metabolic disorder characterised by the excessive excretion of β-alanine, 3-hydroxypropionic acid,R-andS-3-amino- and 3-hydroxyisobutyric acids andS-2-(hydroxymethyl)butyric acid is probably due to deficient activities of malonic, methylmalonic and ethylmalonic semialdehyde dehydrogenases. These dehydrogenation reactions could be mediated by one enzyme, or by enzymes with a common subunit, and bothR- andS-methylmalonic semialdehydes seem to be equally affected. The patient is now aged 4… CONTINUE READING
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