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 micromol/l; mean +/- S.D; n = 26) by means of reversed-phase HPLC method on a C18 column with UV detection.
Krijt
J
J
Institute for Inherited Metabolic Disorders, First Faculty of Medicine, Charles University, Prague, Czech Republic.
Kmoch
S
S
Hartmannová
H
H
Havlícek
V
V
Sebesta
I
I
D000241:Adenosine / Q000031:analogs & derivatives* / Q000134:cerebrospinal fluid; D002648:Child; D002851:Chromatography, High Pressure Liquid; D002855:Chromatography, Thin Layer; D006801:Humans; D013058:Mass Spectrometry; D011686:Purine-Pyrimidine Metabolism, Inborn Errors / Q000134:cerebrospinal fluid; D012015:Reference Standards; D015203:Reproducibility of Results; D013056:Spectrophotometry, Ultraviolet
C043565:succinyladenosine; D000241:Adenosine