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alkaptonuria

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alkaptonuria [al‚kap·tə′nür·ē·ə]
(medicine)
A hereditary metabolic disorder transmitted as an autosomal recessive in humans in which large amounts of homogentisic acid (alkapton) are excreted in the urine due to a deficiency of homogentisic acid oxidase. Also spelled alcaptonuria.


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In 1902, the physician Sir Archibald Garrod, investigating alkaptonuria, a rare inherited enzyme deficiency, suggested that enzymes were important in the detoxification of foreign substances, and that genetically determined differences in the operation of enzymes (characterized by Garrod as "inborn errors of metabolism") could be responsible for ADRs.
Alkaptonuria is an example of the simple model of genetic disease.
 
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