| Article ID | Journal | Published Year | Pages | File Type |
|---|---|---|---|---|
| 8342897 | Molecular Genetics and Metabolism | 2018 | 12 Pages |
Abstract
We proved for the first time that Mutâ/â brain cells are indeed able to produce the characteristic metabolites of MMAuria. We confirmed significant NH4+ accumulation in culture media of Mutâ/â aggregates, suggesting that intracellular NH4+ concentrations might even be higher, gave first clues on the mechanisms leading to NH4+ accumulation in Mutâ/â brain cells, and showed the involvement of neuroinflammatory processes in the neuropathophysiology of MMAuria.
Keywords
PBS2-methylcitrateMethylmalonyl-CoA mutaseLC/MS-MSGLNNT-3IBA1MUTNGFMMAGDHFGFMBPGFAPTHFaKGNH4+ROSα-ketoglutarateAmmoniumNeuroinflammationtetrahydrofolateCNSDIVday in vitroBBBNeurotoxicitycentral nervous systemnerve growth factorfibroblast growth factorBlood-brain barrierCSFCerebrospinal fluidMethylmalonic aciduriaMethylmalonatePhosphate-buffered salineionized calcium binding adaptor molecule 1knock-outneurotrophin-3wild-typeHyperammonemiaGlial fibrillary acidic proteinMyelin basic proteingalactocerebrosideGalCGluglutamateglutamate dehydrogenaseglutamineGlyGlycineReactive oxygen species
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Authors
Noémie Remacle, Patrick Forny, Hong-Phuc Cudré-Cung, Mary Gonzalez-Melo, Sónia do Vale-Pereira, Hugues Henry, Tony Teav, Hector Gallart-Ayala, Olivier Braissant, Matthias Baumgartner, Diana Ballhausen,