Evaluation of urinary acylglycines by electrospray tandem mass spectrometry in mitochondrial energy metabolism defects and organic acidurias.

@article{Bonaf2000EvaluationOU,
  title={Evaluation of urinary acylglycines by electrospray tandem mass spectrometry in mitochondrial energy metabolism defects and organic acidurias.},
  author={Luisa Bonafė and Heinz Troxler and Thomas A. Kuster and Claus Wilhelm Heizmann and Nestor A. Chamoles and Alberto B. Burlina and Nenad Blau},
  journal={Molecular genetics and metabolism},
  year={2000},
  volume={69 4},
  pages={302-11}
}
We analyzed the urinary acylglycine excretion in 26 patients with mitochondrial energy metabolism disorders and in 55 patients with organic acidurias by electrospray tandem mass spectrometry (ESI-MS/MS), monitoring precursor ions of m/z 90. Urinary concentrations of the different acylglycines were quantified using deuterated internal standards. Normal values for the most important acylglycines were established. In MCAD and MAD (neonatal form) deficiencies, typical excretion patterns of urinary… CONTINUE READING

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