Oligodendroglia from ADSL-deficient patient produce SAICAribotide and SAMP
Language English Country United States Media print-electronic
Document type Case Reports, Journal Article, Research Support, Non-U.S. Gov't
PubMed
20674424
DOI
10.1016/j.ymgme.2010.06.014
PII: S1096-7192(10)00234-9
Knihovny.cz E-resources
- MeSH
- Adenosine Monophosphate analogs & derivatives biosynthesis MeSH
- Adenylosuccinate Lyase deficiency MeSH
- Aminoimidazole Carboxamide analogs & derivatives MeSH
- Fatal Outcome MeSH
- Humans MeSH
- Infant, Newborn MeSH
- Oligodendroglia metabolism MeSH
- Ribonucleosides biosynthesis MeSH
- Check Tag
- Humans MeSH
- Male MeSH
- Infant, Newborn MeSH
- Publication type
- Journal Article MeSH
- Case Reports MeSH
- Research Support, Non-U.S. Gov't MeSH
- Names of Substances
- Adenosine Monophosphate MeSH
- Adenylosuccinate Lyase MeSH
- Aminoimidazole Carboxamide MeSH
- Ribonucleosides MeSH
- succinyladenosine monophosphate MeSH Browser
- succinylaminoimidazole carboxamide riboside MeSH Browser
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.
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