Alpha-ketoadipic aciduria: degradation studies with fibroblasts,.

@article{Wendel1975AlphaketoadipicAD,
  title={Alpha-ketoadipic aciduria: degradation studies with fibroblasts,.},
  author={Udo Wendel and Hugo W. Ruediger and Hildegard Przyrembel and Hans Joachim Bremer},
  journal={Clinica chimica acta; international journal of clinical chemistry},
  year={1975},
  volume={58 3},
  pages={
          271-6
        }
}
Observation of one patient with alpha-ketoadipic aciduria initiated degradation studies with radiolabelled lysine metabolites in fibroblasts in order to localise the metabolic defect. Liberation of 14-CO-2 from alpha-D,L-(1-14-C) aminoadipate and alpha-(1-14-C) ketoadipate was considerably less in the patient's fibroblasts than in the patient's fibroblasts than in normal controls, whereas 14-CO-2 production from (1,5-14-C) glutarate was in the normal range. These results indicate a defect in… CONTINUE READING

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α-aminoadipic aciduria: Chemical and enzymatic studies

  • Journal of Inherited Metabolic Disease
  • 1979
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