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David Gray, Senior Specialist Dietitian at Guy’s and St Thomas’…
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Urea cycle disorders are a group of inherited diseases characterised by incomplete ‘metabolism’ or processing of nitrogen. This metabolic fault causes ammonia to build up in the blood, which poisons the brain.
After we eat protein, our bodies break them down into amino acids. As part of this processing, nitrogen is generated as a waste product. A multi-step pathway of reactions known as the urea cycle is responsible for removing this nitrogen from the blood, turning it into urea and excreting it via the kidneys in urine. The steps of the pathway require enzymes and/or ‘co-factors’ (enzyme helpers) that perform a specific task in the overall processing of nitrogen to urea. In individuals with urea cycle disorders, one of the enzymes or an enzyme ‘co-factor’ from the pathway is absent or has reduced activity. The consequence of the deficiency is that the urea cycle produces ammonia instead of urea.
There are six different types of urea cycle disorders. Each is named after the deficient urea cycle enzyme or, in the case of NAGS, a deficient co-factor:
* arginase deficiency (ARG)
* argininosuccinate lyase deficiency (ASL)
* argininosuccinate synthetase deficiency (ASS)
* carbamyl phosphate synthetase deficiency (CPS)
* N-acetylglutamate synthase deficiency (NAGS)
* ornithine transcarbamylase deficiency (OTC)
Animal proteins are found in dairy products, meat, eggs and fish. Proteins are also found in plants including soy, legumes, grains and nuts. The body uses the amino acids from proteins 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. Requirements are higher in childhood as proteins are needed to support growth and development. Certain amino acids must come from the diet, as the body cannot manufacture them itself. These are known as ‘essential’ amino acids. In periods of fasting or illness, the body often switches to use its own proteins, and stored fats, to generate energy.
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