Abstract.
We test the hypothesis that lysinuric protein intolerance (LPI), a rare autosomal recessive defect of cationic amino acid transport, results from the absence of the recently described y+L amino acid transporter. We compare fluxes of lysine (1 µM) into erythrocytes of normal subjects with those of patients homozygous for the LPI mutation. No significant differences in fluxes through system y+L in normal or LPI cells were found, excluding the possibility that system y+L cannot be expressed in patients with LPI. Reasons for supposing that there may be tissue-specific processing of two recently described genes encoding the y+L transporter are discussed. Polymerase chain reaction measurement of expression of these two genes in an erythroleukemic cell line suggests that alternatively there may be an as-yet-unidentified additional member of this gene family.
Similar content being viewed by others
Author information
Authors and Affiliations
Additional information
Received after revision and accepted: 5 November 1999
Electronic Publication
Rights and permissions
About this article
Cite this article
Boyd, C., Deves, R., Laynes, R. et al. Cationic amino acid transport through system y+L in erythrocytes of patients with lysinuric protein intolerance. Pflügers Arch – Eur J Physiol 439, 513–516 (2000). https://doi.org/10.1007/s004249900215
Received:
Issue Date:
DOI: https://doi.org/10.1007/s004249900215