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Ammonia toxicity to the brain
- Source :
- Journal of Inherited Metabolic Disease, Vol. 36, No 4 (2013) pp. 595-612, Journal of Inherited Metabolic Disease, vol. 36, no. 4, pp. 595-612, J Inherit Metab Dis
- Publication Year :
- 2018
-
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.
- Subjects :
- medicine.medical_specialty
Central nervous system
Biology
medicine.disease_cause
Neuroprotection
Nitric oxide
03 medical and health sciences
chemistry.chemical_compound
0302 clinical medicine
Ammonia
Internal medicine
Glutamine synthetase
Genetics
medicine
Animals
Humans
Hyperammonemia
Genetics (clinical)
030304 developmental biology
CIBM-AIT
0303 health sciences
ddc:618
Brain Neoplasms
Brain
medicine.disease
3. Good health
medicine.anatomical_structure
Endocrinology
Neuroprotective Agents
chemistry
Urea cycle
Signal transduction
Neuroscience
030217 neurology & neurosurgery
Oxidative stress
Subjects
Details
- Language :
- English
- ISSN :
- 01418955
- Database :
- OpenAIRE
- Journal :
- Journal of Inherited Metabolic Disease, Vol. 36, No 4 (2013) pp. 595-612, Journal of Inherited Metabolic Disease, vol. 36, no. 4, pp. 595-612, J Inherit Metab Dis
- Accession number :
- edsair.doi.dedup.....7269e4b601ce61aa2e198eeab1088d5f