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Gene Review:

UROC1 - urocanate hydratase 1

Homo sapiens

Synonyms: FLJ31300, HMFN0320, Imidazolonepropionate hydrolase, Urocanase, Urocanate hydratase

Antener, I. et al., Kalafatic, Z. et al., Kessler, D. et al., Hug, D.H. et al.

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Disease relevance of UROC1

Urocanase (EC 4.2.1.49) from Pseudomonas putida was crystallized after removing one of the seven free thiol groups [1].

High impact information on UROC1

Thioglycolate, competitive inhibitor of urocanase [2].
These findings point to the severe impairment of histidase and urocanase, two enzymes regulating the histidine catabolic pathway [3].
Two sisters with a rare inborn error of histidine metabolism resulting from urocanase deficiency are being presented [4].

References

Structure and action of urocanase. Kessler, D., Rétey, J., Schulz, G.E. J. Mol. Biol. (2004) [Pubmed]
Thioglycolate, competitive inhibitor of urocanase. Hug, D.H., O'Donnell, P.S., Hunter, J.K. Biochem. Biophys. Res. Commun. (1978) [Pubmed]
Biochemical study of malnutrition. Part VI: Histidine and its metabolites. Antener, I., Verwilghen, A.M., Van Geert, C., Mauron, J. International journal for vitamin and nutrition research. Internationale Zeitschrift für Vitamin- und Ernährungsforschung. Journal international de vitaminologie et de nutrition. (1983) [Pubmed]
A liver urocanase deficiency. Kalafatic, Z., Lipovac, K., Jezerinac, Z., Juretic, D., Dumic, M., Zurga, B., Res, L. Metab. Clin. Exp. (1980) [Pubmed]

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UROC1	Bos taurus
CELE_Y51H4A.7	Caenorhabditis elegans
UROC1	Canis lupus familiaris
uroc1	Danio rerio
UROC1	Gallus gallus
UROC1	Macaca mulatta
Uroc1	Mus musculus
Uroc1	Rattus norvegicus
uroc1	Xenopus (Silurana) tropicalis

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