UNIGE document Scientific Article - Review
previous document  unige:35588  next document
add to browser collection

Ammonia toxicity to the brain

Braissant, Olivier
Cudalbu, Cristina
Published in Journal of Inherited Metabolic Disease. 2013, vol. 36, no. 4, p. 595-612
Abstract Hyperammonemia can be caused by various acquired or inherited disorders such as urea cycle defects. The brain is much more susceptible to the deleterious effects of ammonium in childhood than in adulthood. Hyperammonemia provokes irreversible damage to the developing central nervous system: cortical atrophy, ventricular enlargement and demyelination lead to cognitive impairment, seizures and cerebral palsy. The mechanisms leading to these severe brain lesions are still not well understood, but recent studies show that ammonium exposure alters several amino acid pathways and neurotransmitter systems, cerebral energy metabolism, nitric oxide synthesis, oxidative stress and signal transduction pathways. All in all, at the cellular level, these are associated with alterations in neuronal differentiation and patterns of cell death. Recent advances in imaging techniques are increasing our understanding of these processes through detailed in vivo longitudinal analysis of neurobiochemical changes associated with hyperammonemia. Further, several potential neuroprotective strategies have been put forward recently, including the use of NMDA receptor antagonists, nitric oxide inhibitors, creatine, acetyl-L-carnitine, CNTF or inhibitors of MAPKs and glutamine synthetase. Magnetic resonance imaging and spectroscopy will ultimately be a powerful tool to measure the effects of these neuroprotective approaches.
PMID: 23109059
Full text
Article (Published version) (885 Kb) - document accessible for UNIGE members only Limited access to UNIGE
Research group Développement du tube digestif chez les vertébrés (900)
(ISO format)
BRAISSANT, Olivier, MCLIN, Valérie Anne, CUDALBU, Cristina. Ammonia toxicity to the brain. In: Journal of Inherited Metabolic Disease, 2013, vol. 36, n° 4, p. 595-612. doi: 10.1007/s10545-012-9546-2 https://archive-ouverte.unige.ch/unige:35588

332 hits



Deposited on : 2014-04-09

Export document
Format :
Citation style :