Abstract
The transport of purines nucleosides and bases through the blood-cerebrospinal fluid (CSF) barrier is incompletely understood. Allopurinol increases plasma purine but should not elevate CSF purines as a result of xanthine oxidase inhibition due to the absence of this enzyme in the central nervous system (CNS). We have examined the effect of allopurinol (5-10 mg/kg/24 h) on plasma and CSF uric acid (UA), hypoxanthine (Hx), xanthine (X) and inosine (Ino) concentrations in 4 patients with HPRT deficiency (less than 1% of normal erytrocyte HPRT levels). Results (means±SEM) were as fallows:
Allopurinol increased plasma Hx, X and Ino concentrations 4-fold, 10-fold and 2.4 fold, respectively, compared to base-line values. The mean absolute increase in CSF Hx and X concentrations during allopurinol treatment was 17.5 μM and 7.7 μM, respectively. These results indicate that Hx and X may be transported to the brain through the blood-brain barrier in HPRT deficiency.
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Jiménez, M., Puig, J., Mateos, F. et al. 67 HYPOXAHTHINE AND XANTHINE TRANSPORT THROUGH THE BLOOD-BRAIN BARRIES IN HYPOXANTHINE PHOSPHORIBOSYL-TRANSFERASE (HPRT) DEFICIENCY. Pediatr Res 24, 122 (1988). https://doi.org/10.1203/00006450-198807000-00091
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DOI: https://doi.org/10.1203/00006450-198807000-00091