Cerebroatrophic Hyperammonemia https://greenmedinfo.com/taxonomy/term/1250/all en Pantothenic acid may have a therapeutic role in sodium benzoate therapy for patients with hyperammonemia. https://greenmedinfo.com/article/pantothenic-acid-may-have-therapeutic-role-sodium-benzoate-therapy-patients-hy PMID:  Pediatr Res. 2000 Sep;48(3):357-9. PMID: 10960503 Abstract Title:  Effect of pantothenic acid on hippurate formation in sodium benzoate-treated HepG2 cells. Abstract:  Inborn errors of urea synthesis result in hyperammonemia. Sodium benzoate (SB) therapy has been beneficial in the treatment of hyperammonemia. It conjugates with glycine to form hippurate, which is then excreted. SB has also been used to treat children with nonketotic hyperglycinemia (NKH), where glycine is removed, on conjugation, as hippurate. In mammalian liver mitochondria, SB is activated by an ATP-dependent reaction to its CoA ester, before conjugation with glycine. Pantothenic acid (PA) is the precursor of CoA. In this investigation, increasing the amounts of PA increased CoA levels in HepG2 cells. It also significantly increased formation of hippurate in SB-treated cells. These findings suggest a beneficial effect of PA on the SB therapy in children with NKH as well as hyperammonemia. https://greenmedinfo.com/article/pantothenic-acid-may-have-therapeutic-role-sodium-benzoate-therapy-patients-hy#comments Cerebroatrophic Hyperammonemia Hyperammonemia Pantothenic Acid (Vitamin B-5) Human Study Sun, 12 Sep 2010 02:58:04 +0000 greenmedinfo 56307 at https://greenmedinfo.com