Glutaric Aciduria Type 1 (GA1)
The ‘building blocks’ of protein are called amino acids. In GA1, due to the lack of an enzyme, the body cannot properly deal with two of the amino acids, lysine and tryptophan. People with GA1 therefore need to restrict the amount of protein they eat.
The management of GA1 may involve the use of protein substitutes. Protein substitutes for GA1 provide all the amino acids found in protein but no lysine and only a small quantity of tryptophan. As such, they provide a source of protein that is free from, or low in, the amino acids that cause problems in GA1. If used, protein substitutes are used alongside the individual’s allowance of regular dietary protein.