Peptiduria presumably caused by aminopeptidase-P deficiency. A new inborn error of metabolism

  title={Peptiduria presumably caused by aminopeptidase-P deficiency. A new inborn error of metabolism},
  author={Nenad Blau and Alois Niederwieser and David H Shmerling},
  journal={Journal of Inherited Metabolic Disease},
Aminopeptidase-P (APP; EC is an exopeptidase highly specific in cleaving the N-terminal imino bond in oligopeptides of an Xaa-Pro-sequence (Yaron and Berger, 1970). Active participation of APP in collagen metabolism, as well as a role in inactivation of biologically active peptides, has been proposed (Fleminger and Yaron, 1983); however its real biological function is still unknown. We report on a 5-month-old boy with epilepsy, and his healthy 5-year-old brother, both of whom were… CONTINUE READING

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