Document Detail

Histidase and histidinemia. Clinical and molecular considerations.
MedLine Citation:
PMID:  1943682     Owner:  NLM     Status:  MEDLINE    
Histidase (histidine ammonia-lyase, EC 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.
R G Taylor; H L Levy; R R McInnes
Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Molecular biology & medicine     Volume:  8     ISSN:  0735-1313     ISO Abbreviation:  Mol. Biol. Med.     Publication Date:  1991 Feb 
Date Detail:
Created Date:  1991-12-17     Completed Date:  1991-12-17     Revised Date:  2009-11-19    
Medline Journal Info:
Nlm Unique ID:  8403879     Medline TA:  Mol Biol Med     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  101-16     Citation Subset:  IM    
Department of Molecular and Medical Genetics, University of Toronto, Canada.
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MeSH Terms
Alanine / analogs & derivatives,  metabolism
Amino Acid Metabolism, Inborn Errors / enzymology*,  genetics*,  metabolism
Amino Acid Sequence
Histidine / blood*,  metabolism
Histidine Ammonia-Lyase / deficiency*,  genetics*
Infant, Newborn
Molecular Sequence Data
Neonatal Screening
Phenylalanine Ammonia-Lyase / genetics
Skin / enzymology
Urocanic Acid / metabolism
Reg. No./Substance:
104-98-3/Urocanic Acid; 1948-56-7/dehydroalanine; 56-41-7/Alanine; 71-00-1/Histidine; EC Ammonia-Lyase; EC Ammonia-Lyase

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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