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 (PBL(LN)) and from controls (PBL(C)). Adenosine transport was significantly lower in PBL(LN) when compared with that in PBL(C) and a significantly lower number of high affinity sites for [(3)H]nitrobenzylthioinosine binding were quantified per cell ( B (max)) in PBL(LN) when compared with that in PBL(C). After incubation with 25 microM hypoxanthine, adenosine transport was significantly decreased in PBL(LN) with respect to PBL(C). Hypoxanthine incubation lowers [(3)H]nitrobenzylthioinosine binding in PBL(C), with respect to basal conditions, but does not affect it in PBL(LN). This indicates that hypoxanthine affects adenosine transport in control and hypoxanthine-guanine phosphoribosyltransferase-deficient cells by different mechanisms.
Clinical Biochemistry Division, 'La Paz' University Hospital, Paseo de la Castellana 261, 28046 Madrid, Spain. rtorres.hulp@salud.madrid.org
Rosa J
RJ
Torres
Isabel
I
Deantonio
Carmen
C
Prior
Juan G
JG
Puig
D000241:Adenosine / Q000378:metabolism*; D001692:Biological Transport / Q000187:drug effects / Q000502:physiology; D002478:Cells, Cultured; D000242:Cyclic AMP / Q000378:metabolism; D006801:Humans; D019271:Hypoxanthine / Q000378:metabolism / Q000494:pharmacology; D007041:Hypoxanthine Phosphoribosyltransferase / Q000172:deficiency / Q000502:physiology; D007926:Lesch-Nyhan Syndrome / Q000097:blood / Q000201:enzymology / Q000378:metabolism* / Q000473:pathology; D008214:Lymphocytes / Q000737:chemistry / Q000201:enzymology / Q000378:metabolism*; D013868:Thioinosine / Q000031:analogs & derivatives* / Q000378:metabolism
D019271:Hypoxanthine; D013868:Thioinosine; D000242:Cyclic AMP; D007041:Hypoxanthine Phosphoribosyltransferase; C001789:4-nitrobenzylthioinosine; D000241:Adenosine