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Glutaric aciduria means ‘glutaric acid in the urine’. This inherited metabolic condition is also known as glutaric acidaemia – or ‘glutaric acid in the blood’. It normally manifests in infancy and early childhood. There are two distinct forms.
In type I glutaric aciduria, abnormal processing or ‘metabolism’ of lysine, hydroxylysine and tryptophan generates toxic by-products that cause severe brain damage. Lysine, hydroxylysine and tryptophan are amino acids – the building blocks of proteins. After eating proteins, the body breaks them down into amino acids. Animal proteins include dairy products, meat, eggs and fish. Proteins are also found in plants including soy, legumes, grains and nuts. The body uses the amino acids to make its own proteins essential for life – for example enzymes; structural proteins in muscles, hair, skin, cells and cartilage; proteins that generate movement in muscles; or those involved in cell functioning or immune responses. In periods of starvation, amino acids can be redirected to generate energy for the body. When lysine, hydroxylysine and tryptophan are not metabolised properly, intermediate breakdown products (glutaric acid, glutaryl-CoA, 3-hydroxyglutaric acid and glutaconic acid) accumulate in body fluids and brain tissue. The build-up of metabolites is especially pronounced when the body is under stress. Characteristically, between the ages of 6 and 18 months the child experiences a metabolic ‘crisis’. This sudden episode occurs during periods of illness or fever or after immunisation, lack of food or minor head injury. The crisis seems to be a turning point in the condition and triggers significant brain damage. The basal ganglia of the brain, which helps control movement, is most affected. In addition, the metabolites cause damage to other organs, although to a lesser extent. It is not known how glutaric acid and the other metabolites exert their destruction. The risk of crises diminishes as the child gets older.
Type II glutaric aciduria is linked to a deficiency in enzymes involved in processing fats and proteins. Partly metabolised fats and proteins accumulate in the body and cause the blood and tissues to become dangerously acidic. Like type I, metabolic crises triggered by common childhood illnesses or stress worsen the condition. In this scenario they trigger the body to break down its own stores of protein and fats thus generating the toxic by-products that cause the body harm. Liver and brain damage are characteristic.
The biochemical alterations in both types of glutaric aciduria can hamper the production of carnitine, a product of lysine metabolism important for generating energy from dietary fats.
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