| Histidase and histidinemia. Clinical and molecular considerations. | |
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MedLine Citation:
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PMID: 1943682 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Histidase (histidine ammonia-lyase, EC 4.3.1.3) catalyzes the deamination of L-histidine to trans-urocanic acid in the liver and skin of mammals. Histidase deficiency results in increased histidine and histamine in blood, and decreased urocanic acid in blood and skin. In this review we discuss current research on: (1) the mechanism of formation of an unusual residue, dehydroalanine, at the active site of histidase; and (2) the role of urocanic acid as an ultraviolet light-induced immunoregulator in the skin, and the implications of urocanic acid deficiency for human histidinemia. Genetic mechanisms that may account for the 1% of histidinemic patients with neurological impairments are considered briefly. |
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Authors:
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R G Taylor; H L Levy; R R McInnes |
Publication Detail:
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Type: Journal Article; Review |
Journal Detail:
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Title: Molecular biology & medicine Volume: 8 ISSN: 0735-1313 ISO Abbreviation: Mol. Biol. Med. Publication Date: 1991 Feb |
Date Detail:
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Created Date: 1991-12-17 Completed Date: 1991-12-17 Revised Date: 2009-11-19 |
Medline Journal Info:
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Nlm Unique ID: 8403879 Medline TA: Mol Biol Med Country: ENGLAND |
Other Details:
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Languages: eng Pagination: 101-16 Citation Subset: IM |
Affiliation:
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Department of Molecular and Medical Genetics, University of Toronto, Canada. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Alanine
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analogs & derivatives,
metabolism Amino Acid Metabolism, Inborn Errors / enzymology*, genetics*, metabolism Amino Acid Sequence Animals Histidine / blood*, metabolism Histidine Ammonia-Lyase / deficiency*, genetics* Humans Infant, Newborn Molecular Sequence Data Neonatal Screening Phenylalanine Ammonia-Lyase / genetics Skin / enzymology Urocanic Acid / metabolism |
| Chemical | |
Reg. No./Substance:
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104-98-3/Urocanic Acid; 1948-56-7/dehydroalanine; 56-41-7/Alanine; 71-00-1/Histidine; EC 4.3.1.24/Phenylalanine Ammonia-Lyase; EC 4.3.1.3/Histidine Ammonia-Lyase |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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