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Combined iminoglycinuria and cystine- and dibasic aminoaciduria in patients with propionic acidaemia and 3-methylcrotonylglycinuria.

Abstract
Urinary amino acids have been determined in six patients with propionic acidaemia, one of whom also showed 3-methylcrotonylglycinuria. Two patients, including the subject with 3-methylcrotonylglycinuria, showed a gross aminoaciduria with features of both cystinuria and iminoglycinuria. We suggest a defect in certain amino acid transport systems in some patients with these disorders.
AuthorsP Purkiss, R A Chalmers, O Borud
JournalJournal of inherited metabolic disease (J Inherit Metab Dis) Vol. 3 Issue 3 Pg. 85-6 ( 1980) ISSN: 0141-8955 [Print] United States
PMID6775144 (Publication Type: Journal Article)
Chemical References
  • Amino Acids
  • Amino Acids, Diamino
  • Crotonates
  • Propionates
  • beta-methylcrotonylglycine
  • Glycine
Topics
  • Amino Acid Metabolism, Inborn Errors (urine)
  • Amino Acids (urine)
  • Amino Acids, Diamino (urine)
  • Child
  • Child, Preschool
  • Crotonates (urine)
  • Cystinuria
  • Glycine (analogs & derivatives, urine)
  • Humans
  • Infant
  • Infant, Newborn
  • Infant, Newborn, Diseases (urine)
  • Propionates (blood)

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