Hyperammonemia
See also: Ammonia Toxicity
Defects in Urea Cycle and Hyperammonemia
Hyperammonemia (or hyperammonaemia) is a metabolic disturbance characterized by an excess of ammonia in the blood. It is a dangerous condition that may lead to encephalopathy and death. It may be primary or secondary.
Ammonia is a substance that contains nitrogen. It is a product of the catabolism of protein. It is converted to the less toxic substance urea prior to excretion in urine by the kidneys. The metabolic pathways that synthesize urea involve reactions that start in the mitochondria and then move into thecytosol. The process is known as the urea cycle, which comprises several enzymes acting in sequence.
ARTICLES/STUDIES
Acetyl-L-carnitine lowers hyperammonemia less markedly thanL-carnitine.