Urocanic acid is an intermediate in the catabolism

Catabolism

Catabolism is the set of metabolic pathways that break down molecules into smaller units and release energy. In catabolism, large molecules such as polysaccharides, lipids, nucleic acids and proteins are broken down into smaller units such as monosaccharides, fatty acids, nucleotides, and amino...

 of L-histidine

Histidine

Histidine Histidine, an essential amino acid, has a positively charged imidazole functional group. It is one of the 22 proteinogenic amino acids. Its codons are CAU and CAC. Histidine was first isolated by German physician Albrecht Kossel in 1896. Histidine is an essential amino acid in humans...

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Metabolism

It is formed from L-histidine through the action of histidine ammonialyase (also known as histidase or histidinase) by elimination of ammonium.

In the liver, urocanic acid is transformed by urocanate hydratase

Urocanate hydratase

Urocanase is the enzyme that catalyzes the second step in the degradation of histidine, the hydration of urocanate into imidazolonepropionate....

 (or urocanase) to 4-imidazolone-5-propionic acid and subsequently to glutamic acid

Glutamic acid

Glutamic acid is one of the 20 proteinogenic amino acids, and its codons are GAA and GAG. It is a non-essential amino acid. The carboxylate anions and salts of glutamic acid are known as glutamates...

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Clinical significance

Inherited deficiency of urocanase leads to elevated levels of urocanic acid in the urine, a condition known as urocanic aciduria

Urocanic aciduria

Urocanic aciduria, also called urocanate hydratase deficiency or urocanase deficiency, is an autosomal recessive metabolic disorder caused by a deficiency of the enzyme urocanase. It is a secondary disorder of histidine metabolism.-Pathophysiology:...

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Function

Urocanic acid was detected in animal sweat and skin where, among other possible functions, it acts as an endogenous sunscreen or photoprotectant against UVB-induced DNA damage. Urocanic acid is found predominantly in the stratum corneum

Stratum corneum

The stratum corneum is the outermost layer of the epidermis, consisting of dead cells that lack nuclei and organelles. The purpose of the stratum corneum is to form a barrier to protect underlying tissue from infection, dehydration, chemicals and mechanical stress...

 of the skin and it is likely that most of it is derived from filaggrin

Filaggrin

Filaggrin is a filament-associated protein that binds to keratin fibers in epithelial cells.-Profilaggrin:Filaggrin monomers are tandemly clustered into a large, 350kDa protein precursor known as profilaggrin. In the epidermis, these structures are present in the keratohyalin granules in cells of...

 catabolism (a histidine-rich protein). When exposed to UVB irradiation, trans-urocanic acid is converted in vitro and in vivo to the cis isomer. The cis form is known to activate suppressor T cells.

History

Urocanic acid was first isolated in 1874 by the chemist M. Jaffé from the urine of a dog, hence the name ( = urine, and canis = dog).

External links

• The Online Metabolic and Molecular Bases of Inherited Disease - Chapter 80 - An overview of disorders of histidine metabolism, including urocanic aciduria.