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David Gray, Senior Specialist Dietitian at Guy’s and St Thomas’…
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Maple syrup urine disease is a genetic disease characterised by incomplete metabolism of the amino acids leucine, isoleucine and valine. It owes its unusual name to the characteristic sweet-smelling urine of those affected.
In individuals with this disease, poor processing of leucine, isoleucine and valine causes a build up of these molecules and their by-products in the blood and urine. Untreated, this can cause seizures, severe brain damage and coma. Few infants survive without intervention. The condition shows similarities to isovaleric acidaemia, in which leucine metabolism is affected.
Amino acids are the building blocks of proteins. After eating proteins, the body ‘metabolises’ or 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 fasting or illness, the body often switches to use its own proteins to generate energy.
Leucine, isoleucine and valine are essential amino acids meaning that the body cannot make them. Therefore, these amino acids come from ingested protein or from the breakdown of previously ingested and stored proteins. Leucine, isoleucine and valine are classified as ‘branched amino acids’, which describes their specific chemical composition.
Another name for maple syrup urine disease is branched-chain ketoaciduria. This describes the presence in the urine of keto acids. These are metabolic by-products created when the body resorts to using its own protein and fat stores for energy.
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