Proprionic Acidemia (PROP) is a genetic disorder caused by a deficiency in the propionyl-CoA carboxylase enzyme. Read more on PROP via this link.

Propionyl-CoA carboxylase is responsible for the break down of the amino acids): isoleucine (Ile), valine (Val), methionine (Met) and threonine (Thr).

Ile, Val, Met and Thr are important amino acids that we need to eat from protein in foods. Protein is important for growth in children and health maintenance in adults.

Propionyl-CoA carboxylase is an enzyme that helps break down Propionyl-CoA.

The symptoms of PROP usually appear within the first few days of life. These symptoms may include worsening: poor appetite, vomiting, irritability, low muscle tone and lack of energy.

Long term symptoms of this disorder include pancreatitis, seizures and developmental delay.

Patients are prescribed a protein-restricted diet to prevent high levels of propionyl-CoA.

People living with PROP are prescribed their daily protein restriction by their doctor or dietitian. Patients also frequently restrict their Ile, Val, Met and Thr intake. These daily goals often need to be changed based on each person’s nutritional needs and age requirements.

Since nutrition labels on foods do not list Ile, Val, Met and Thr content of foods, it is very difficult for families to track their daily dietary intake.

We hope this App makes daily protein and amino acid counting and meal planning easier for people following low protein diets.