disodium citrate

Disodium citrate is a small molecule nutritional supplement being investigated as an anaplerotic therapy for the treatment of propionic acidemia. Propionic acidemia is a metabolic disorder caused by a deficiency in the enzyme propionyl-CoA carboxylase, which leads to an impairment of the citric acid (Krebs) cycle. Disodium citrate works by replenishing intermediates in the Krebs cycle, specifically increasing the urinary excretion of alpha-ketoglutarate, succinate, and fumarate, thereby bypassing the metabolic block. In clinical studies led by Nicola Longo at the University of Utah, disodium citrate supplementation was found to be safe and associated with favorable biochemical changes and a potential reduction in hospitalizations for patients with this condition.

Other names

disodium hydrogen citratesodium acid citratecitrate