The content you are trying to access is intended
for Healthcare Professionals only.

Are you a healthcare professional?
The following content is restricted for healthcare professionals only.
You will be redirected to our patient websites.
The following content is for patients
You are being directed to our healthcare professional website.
Maple Syrup Urine Disease (MSUD) | Protein metabolism | Inborn errors of metabolism

Maple Syrup Urine Disease (MSUD)

Maple Syrup Urine Disease (MSUD)

The ‘building blocks’ of protein are called amino acids. In MSUD, due to the lack of an enzyme, the body cannot properly deal with three amino acids, leucine, isoleucine and valine - collectively known as the branched chain amino acids (BCAAs). People with MSUD therefore need to severely restrict the amount of protein they eat.


A key element in the management of MSUD is the use of protein substitutes. Protein substitutes for MSUD provide all the amino acids found in protein but no leucine, isoleucine or valine. In combination with the small allowance of regular dietary protein, protein substitutes ensure that an individual with MSUD has an adequate intake of protein.


Our product range for MSUD

Our support product range 

Inborn Errors of Metabolism