Adenosine transport in peripheral blood lymphocytes from Lesch-Nyhan patients.

We postulated that adenosine function could be related to some of the neurological features of Lesch-Nyhan syndrome and therefore characterized adenosine transport in PBLs (peripheral blood lymphocytes) obtained from Lesch-Nyhan patients (PBLLN) and from controls (PBLC). Adenosine transport was significantly lower in PBLLN when compared with that in PBLC and a significantly lower number of high affinity sites for [H-3]nitrobenzylthioinosine binding were quantified per cell (B-max) in PBLLN when compared with that in PBLC. After incubation with 25 muM hypoxanthine, adenosine transport was significantly decreased in PBLLN with respect to PBLC. Hypoxanthine incubation lowers [H-3]nitrobenzylthioinosine binding in PBLC, with respect to basal conditions, but does not affect it in PBLN. This indicates that hypoxanthine affects adenosine transport in control and hypoxanthine-guanine phosphoribosyltransferase-deficient cells by different mechanisms.