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Oral administration of epsilon N-acetyllysine and homocitrulline in lysinuric protein intolerance.
J Pediatr ; 102(3): 388-90, 1983 Mar.
Article em En | MEDLINE | ID: mdl-6402575
The defect of epithelial diamino acid transport in lysinuric protein intolerance (LPI) results from an abnormality of the basolateral cell membranes. Therefore the lysine deficiency, which is one of the consequences of this defect, cannot be corrected by oral administration of lysine, either in free or peptide form. In search of useful lysine derivatives, we studied in patients with LPI the absorption after oral administration of homocitrulline and epsilon N-acetyllysine. Acetyllysine, but not homocitrulline, normalized the subnormal plasma lysine concentrations.
Assuntos
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Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Citrulina / Erros Inatos do Metabolismo dos Aminoácidos / Lisina Limite: Adolescent / Adult / Child / Child, preschool / Female / Humans / Male Idioma: En Revista: J Pediatr Ano de publicação: 1983 Tipo de documento: Article País de publicação: Estados Unidos
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Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Citrulina / Erros Inatos do Metabolismo dos Aminoácidos / Lisina Limite: Adolescent / Adult / Child / Child, preschool / Female / Humans / Male Idioma: En Revista: J Pediatr Ano de publicação: 1983 Tipo de documento: Article País de publicação: Estados Unidos