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H(2)O(2) increases de novo synthesis of (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin via GTP cyclohydrolase I and its feedback regulatory protein in vitiligo.
J Inherit Metab Dis
February 2009
Clinical and Experimental Dermatology/Department of Biomedical Sciences, University of Bradford, Bradford, UK.
Patients with vitiligo accumulate up to 10(-3) mol/L concentrations of H(2)O(2) in their epidermis, which in turn affects many metabolic pathways in this compartment, including the synthesis and recycling of the cofactor (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin (6BH(4)). De novo synthesis of 6BH(4) is dependent on the rate-limiting enzyme GTP cyclohydrolase I (GTPCHI) together with its feedback regulatory protein (GFRP). This step is controlled by 6BH(4) and the essential amino acid L-phenylalanine.
View Article and Find Full Text PDFVitiligo is not caused by mutations in GTP-cyclohydrolase I gene.
Clin Exp Dermatol
March 2000
Department of Dermatology, Calcutta School of Tropical Medicine, India.
GTP-cyclohydrolase I (GTP-CH I) is the initial and rate-limiting enzyme in the synthesis of tetrahydrobiopterin, which regulates melanin biosynthesis. GTP-CH I is therefore a candidate gene for vitiligo. We have carried out DNA sequencing of all six exons and the promoter region of this gene, comprising 1759 base pairs, in 25 nonsyndromic vitiligo patients.
View Article and Find Full Text PDFIn vivo evidence for compromised phenylalanine metabolism in vitiligo.
Biochem Biophys Res Commun
February 1998
Department of Biomedical Sciences, University of Bradford, West Yorkshire, United Kingdom.
Human epidermal melanocytes and keratinocytes express mRNA for all enzymes involved in de novo synthesis/recycling of the cofactor (6R) L-erythro 5,6,7,8 tetrahydrobiopterin (6BH4) in normal healthy individuals. An enhanced epidermal de novo synthesis was identified in association with decreased epidermal phenylalanine hydroxylase and 4a carbinolamine dehydratase in patients with vitiligo. The latter event leads to an accumulation of the nonenzymatic isomer (7R) L-erythro 5,6,7,8 tetrahydrobiopterin (7BH4) inhibiting phenylalanine hydroxylase (PAH) with an apparent Ki = 10(-6) M.
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