Propionic Acidemia (PROP)



Propionic acidemia (PROP) is an inherited condition in which the body is unable to break down certain proteins and fats, which can lead to a harmful amount of organic acids and toxins in the body. If left untreated, PROP can cause brain defects or even death. However, if this condition is identified early and proper treatment is received, some complications of PROP can be minimized. Signs of PROP usually start within a few days after birth. Early signs of PROP include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), sleeping longer or more often, and tiredness.  Symptoms can be triggered by long periods of time without eating, illnesses, and infections. In some cases, signs may not appear until childhood or later.

Condition Type:

Propionic acidemia (PROP) affects 1 out of every 35,000 to 75,000 babies born in the United States.

More Information for Parents:
Also known as:
  • PROP
  • Ketotic glycinemia
  • Ketotic hyperglycinemia
  • PCC deficiency
  • Propionyl-CoA carboxylase deficiency

Core Conditions