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Title: Blue People of Troublesome Creek
Deposited By: Paul Beaumont, Science and Plants for Schools
Date Deposited: 20 October 2003
Description: Oxidation of the iron atom in haemoglobin (Hb) leads to the formation of methaemoglobin (MetHb) which is unable to transport oxygen. The enzyme diaphorase is able to reduce the iron in MetHb and thereby regenerate Hb. The so-called ‘Blue People of Troublesome Creek’ suffer from a recessive genetic disorder characterised by a failure of diaphorase production or function. This condition leads to the accumulation of MetHb with consequent blue colouration of the skin. As a result of their condition, many of the Blue People were shunned by society and this led to a number of consanguineous marriages with the result that the disorder was confined to a few families. The treatment for the condition is to take methylene blue and this in itself seems perverse - a blue dye to treat a blue condition! Essentially the methylene blue is reduced to a colourless form on ingestion and this reduced form reacts with MetHb and converts it back to Hb regenerating methylene blue. At some point all the methylene blue is in the reduced form (i.e. when there is no MetHb left) and is excreted through the urine. At the point of exit it is colourless but reacts with oxygen in the atmosphere to produce blue urine - the main side-effect of the treatment.
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