Amino acids are an essential component of protein, which are complex molecules that play important roles in the structure and function of cells. Disorders of amino acid absorption can stop amino acids from making their way into your cells, or they can impede your body’s process for breaking down amino acids. These disorders are often inherited, which means they will be present from birth.
There are many amino acids that play crucial roles in bodily function, and there are multiple types of amino acid absorption disorders as a result. These disorders include:
The type of amino acid disorder you have dictates the treatment you will receive.
With phenylketonuria, which causes an amino acid called phenylalanine to accumulate in the body, reducing the intake of foods containing this amino acid is key. Patients will also need protein supplements to make up for their consumption of the nutrient, and some people benefit from a medication called sapropterin, which can sometimes allow a person to consume foods that would otherwise be dangerous with their condition.
As for maple syrup urine disease, this condition results from the body’s inability to break down leucine, valine, and isoleucine, which are common amino acids. Primary treatment entails food restriction, but supplemental isoleucine and valine, along with thiamine (also known as vitamin B1), may be necessary to ensure a person receives essential nutrients. Additionally, maple syrup urine disease requires lifelong monitoring to prevent a more serious metabolic crisis.
Other disorders will entail a different approach when it comes to medication and nutrient supplementation. Your doctor can help you develop a nutrition plan that supports your health considering your amino acid intolerance.