Urocanic
Urocanic acid is a compound that is involved in the metabolism of histidine, an amino acid found in proteins. It is formed from histidine by the enzyme histidine ammonia-lyase, also known as histidase. Urocanic acid can be further metabolized to formiminoglutamic acid by the enzyme urocanase.
Structure and properties[edit | edit source]
Urocanic acid is a unsaturated carboxylic acid. It has a cyclic structure, with a five-membered ring that includes a double bond and a carboxyl group. The double bond gives urocanic acid its unsaturated character. The carboxyl group makes it an acid.
Role in histidine metabolism[edit | edit source]
In the metabolism of histidine, urocanic acid plays a key role. Histidine is first converted to urocanic acid by the enzyme histidase. This reaction involves the removal of an ammonia molecule from histidine. The urocanic acid can then be further metabolized to formiminoglutamic acid by the enzyme urocanase. This reaction involves the addition of a water molecule to the urocanic acid.
Clinical significance[edit | edit source]
Abnormal levels of urocanic acid can be a sign of a disorder in histidine metabolism. For example, in histidinemia, a rare genetic disorder, there is an excess of histidine and urocanic acid in the blood. This is due to a deficiency of the enzyme histidase.
See also[edit | edit source]
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