Abstract
Cyclic nucleotides in the central nervous system may have “first” messenger roles as well as the classic “second” messenger effects. Such primary actions may include regulation of neurotransmitter synthesis, control of certain cellular movements and effects on both tropic and developmental processes. Low cyclic AMP (cAMP) levels are reported in spinal fluids (CSF) from patients with certain basal ganglion diseases, i.e. Parkinson's and advanced Huntington's disease. Cyclic GMP (cGMP) in the CSF is felt to reflect central cholinergic activity. In an effort to find a suitable biochemical marker of disease activity in the Lesch-Nyhan syndrome (L-NS) we made serial measurements of cAMP and cGMP on deproteinized CSF extracts using a radioimmunoassay. Three boys with L-NS at ages 29, 68, and 128 months had 5 CSF determinations over the subsequent 40 months period. Seventeen age-matched patients undergoing leukemia workup and showing no evidence of CNS disease were used as controls. The control group had values of 1.4 ± 0.6 pmol/ml for cAMP and 0.1 ± 0.05 for cGMP. The L-NS subjects had cAMP level of 4.3 ± 2.1 and cGMP of 1.1 ± 0.7. The correlation (Pearson's) between cAMP and cGMP levels in the L-NS subjects was r = 0.83 and in the controls only 0.26. These findings raise the possibility of an as yet undescribed CSF metabolite with caffeine-like activity in the L-NS spinal fluid that may block cyclic nucleotide phosphodiesterase activity and lead to CSF cyclic nucleotide elevations.
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Laurence Edwards, N., Johnston, M. & Silverstein, F. 28 CEREBROSPINAL FLUID CYCLIC NUCLEOTIDE ALTERATIONS IN THE LESCH-NYHAN SYNDROME. Pediatr Res 24, 115 (1988). https://doi.org/10.1203/00006450-198807000-00052
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DOI: https://doi.org/10.1203/00006450-198807000-00052