Introduction

Glutaric aciduria type 1 (GA1) is a rare but serious inherited condition. It means the body cant process certain amino acids ("building blocks" of protein), causing a harmful build-up of substances in the blood and urine.

Normally, our bodies break down protein foods like meat and fish into amino acids. Any amino acids that aren't needed are usually broken down and removed from the body.

Babies with GA1 are unable to break down the amino acids lysine, hydroxylysine and tryptophan.

Normally, these amino acids are broken down into a substance called glutaric acid, which is then converted into energy. Babies withGA1 dont have the enzyme that breaks down glutaric acid, leading to a harmfully high level of this and other substances in the body.

Content supplied by the NHS Website

Medically Reviewed by a doctor on 28 Nov 2016