Identification and determination of succinyladenosine in human cerebrospinal fluid

Succinyladenosine (S-Ado) is a biochemical marker of adenylosuccinase deficiency - the genetic defect of purine de novo synthesis. S-Ado has been previously reported as normally undetectable in cerebrospinal fluid (CSF) of children not suffering from this defect. In present study, we employed solid-phase extraction and thin-layer chromatography for isolation of a compound with spectral and chromatographic characteristics identical to S-Ado from human CSF. The high performance liquid chromatography-negative-ion electrospray ionization mass spectrometry analysis confirmed that the isolated compound is S-Ado. We established the reference values of S-Ado in CSF of children (1.1 +/- 0.4 mu mol/l; mean +/- S.D; n = 26) by means of reversed-phase HPLC method on a C-18 column with UV detection. (C) 1999 Elsevier Science B.V. All rights reserved.