PMID: 6402575Mar 1, 1983Paper

Oral administration of epsilon N-acetyllysine and homocitrulline in lysinuric protein intolerance

The Journal of Pediatrics
J RajantieJ Perheentupa

Abstract

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.

References

Jun 1, 1980·The Journal of Clinical Investigation·J F DesjeuxJ Perheentupa
Apr 1, 1981·The Journal of Clinical Investigation·J RajantieJ Perheentupa

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Citations

Feb 1, 1984·European Journal of Pediatrics·I GotoY Kuroiwa
Aug 2, 2007·Journal of Applied Genetics·Loreta CimbalistieneVaidutis Kucinskas
Nov 1, 1984·Current Problems in Pediatrics·M L Batshaw
Mar 31, 2004·Molecular Genetics and Metabolism·Manuel PalacínAntonio Zorzano
Nov 23, 2016·Chemical Communications : Chem Comm·Laurent VialJulien Leclaire

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