OAc Concept

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.


Dr. Schär Deutschland GmbH 

Dieselstrasse 23
61191 Rosbach v. d. H. · DE

Tel. +49 6003 9117 23
Hotline DE: 0800 153 153-3
Hotline AT: 0800 500 144




Aminosäurenmischung | diätetische Behandlung von Phenylketonurie | erstes Lebensjahr | Erwachsene | glutenfrei | HPA | Hyperphenylalaninämie | Jugendliche | Kinder | Kleinkinder | Kohlenhydrate | Mineralstoffe | phenylalaninfreie | Proteinsupplemente | Phenylketonurie | PKU | Schwangere |Spurenelemente | Säuglinge | Vitamine


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