The dietary management dietary management consists of a diet low in Methionine, Valine, Threonine and Isoleucine (i.e. native protein), which are metabolic precursors of propionyl CoA. Such low protein diet requires the addition of protein supplements free from these amino acids.
Organic Acidemias / Acidurias
Organic acidemias are inherited metabolic disorders, related to the metabolic pathways of amino acids, carbohydrates, and fatty acids. The most common organic acidemias are propionic acidemia (PPA) and methylmalonic acidemia (MMA). Enzyme defects in degradation of propionyl CoA or methylmalonyl CoA lead to accumulation of these metabolites in the body. The severity of symptoms varies widely, ranging from mild neurological disturbances to metabolic crisis and even death.