“…Acetylcarnitine is also involved in the beta-oxidation of fatty acids, which facilitates the translocation of heavy acyl-CoA residues from the cytoplasm to the mitochondrial matrix, where beta-oxidation and energy production take place. Disturbance in transportation results in the accumulation of unoxidized fatty acyl-CoA molecules in the cytosol that are believed to hamper the urea cycle, leading to ammonia accumulation. , Similarly, glutamate, which is also responsible for ammonia transportation, eventually causes hyperammonemia when it is in a lower concentration in the blood. Yelamanchi et al explained that deficiency of glutamate and the enzymes responsible for its metabolism are the main causes of hyperammonemia.…”