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Biochem J ; 377(Pt 3): 733-9, 2004 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-14572307

RESUMO

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.


Assuntos
Adenosina/metabolismo , Síndrome de Lesch-Nyhan/metabolismo , Linfócitos/metabolismo , Tioinosina/análogos & derivados , Transporte Biológico/efeitos dos fármacos , Transporte Biológico/fisiologia , Células Cultivadas , AMP Cíclico/metabolismo , Humanos , Hipoxantina/metabolismo , Hipoxantina/farmacologia , Hipoxantina Fosforribosiltransferase/deficiência , Hipoxantina Fosforribosiltransferase/fisiologia , Síndrome de Lesch-Nyhan/sangue , Síndrome de Lesch-Nyhan/enzimologia , Síndrome de Lesch-Nyhan/patologia , Linfócitos/química , Linfócitos/enzimologia , Tioinosina/metabolismo
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