Succinylpurines accumulate in the body fluids of patients with adenylosuccinate lyase (ADSL) deficiency but their source in the cerebrospinal fluid remains obscure. Study based on the incorporation of 13C-stable isotope-labeled glycine into cultured oligodendroglia from ADSL-deficient patient and the measurement of labeled products by LC/MS/MS showed total intracellular concentrations of succinylpurines from 45 to 99μmol/l and so these results suggest that these cells can be the source of the compounds in vivo.
- MeSH
- adenosinmonofosfát analogy a deriváty biosyntéza MeSH
- adenylsukcinátlyasa nedostatek MeSH
- aminoimidazolkarboxamid analogy a deriváty MeSH
- fatální výsledek MeSH
- lidé MeSH
- novorozenec MeSH
- oligodendroglie metabolismus MeSH
- ribonukleosidy biosyntéza MeSH
- Check Tag
- lidé MeSH
- mužské pohlaví MeSH
- novorozenec MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- práce podpořená grantem MeSH
- Názvy látek
- adenosinmonofosfát MeSH
- adenylsukcinátlyasa MeSH
- aminoimidazolkarboxamid MeSH
- ribonukleosidy MeSH
- succinyladenosine monophosphate MeSH Prohlížeč
- succinylaminoimidazole carboxamide riboside MeSH Prohlížeč
Citrobacter freundii was grown aerobically in a chemostat on a mineral medium with galactose or glucose as carbon and energy sources under limitation by carbon or nitrogen source respectively. At various specific growth rates ranging from 7 to 95% mumax the culture in steady state was analysed and growth yield, specific metabolic rate of substrate utilization, intracellular concentration of pyruvate, ATP, ADP, AMP and energy charge were determined and plotted as functions of dilution rate. In all four types of experiments the physiological state of cells remained practically independent of dilution rate up to D=0.6 mumax, and at a given specific growth rate nearly independent on mumax and type of limitation. At approximately D=0.6 mumax, which is close to the maximum output dilution rate Dm, the physiological state of the cells changed: growth yields decreased and intr cellular pyruvate and adenylates concentrations increased. Consequently, in a given medium two dilution rates exist at which growth rate dx/dt is the same but the physiology of the population is quite different.
- MeSH
- adenosindifosfát biosyntéza MeSH
- adenosinmonofosfát biosyntéza MeSH
- adenosintrifosfát biosyntéza MeSH
- aerobióza MeSH
- chlorid amonný metabolismus MeSH
- Citrobacter růst a vývoj metabolismus MeSH
- energetický metabolismus MeSH
- Enterobacteriaceae metabolismus MeSH
- galaktosa metabolismus MeSH
- glukosa metabolismus MeSH
- pyruváty biosyntéza MeSH
- Publikační typ
- časopisecké články MeSH
- Názvy látek
- adenosindifosfát MeSH
- adenosinmonofosfát MeSH
- adenosintrifosfát MeSH
- chlorid amonný MeSH
- galaktosa MeSH
- glukosa MeSH
- pyruváty MeSH
