title: Regulation of metabolism of retinol-binding protein by vitamin A status in children with biliary atresia.
abstract: Regulation of retinol-binding protein (RBP) by vitamin A status was studied in 43 children; 25 had biliary atresia and vitamin A deficiency, 15 had biliary atresia treated by vitamin A, and 9 control children had normal liver and vitamin A status. Vitamin A and RBP were assayed and the two forms of RBP, holo-RBP and apo-RBP, were separated in both liver and plasma. No difference in liver RBP concentrations was found between the three groups; apo-RBP was the most abundant form. Plasma RBP concentrations and the ratio of retinol to RBP were lower for vitamin A-deficient than for vitamin A-treated children. Two models could be proposed: 1) a preferential secretion of holo-RBP with variations in RBP catabolism or synthesis in vitamin A-deficient liver and 2) a continuous secretion of RBP by the liver with a rapid clearance of plasma apo-RBP in vitamin A deficiency.
authors.affiliation: Unité de recherche d'Hépatologie INSERM U 56, LeKremlin-Bicêtre, France.
authors.forename: M S
authors.initials: MS
authors.lastname: Mourey
authors.forename: G
authors.initials: G
authors.lastname: Siegenthaler
authors.forename: O
authors.initials: O
authors.lastname: Amédée-Manesme
mesh_terms: D001656:Biliary Atresia / Q000150:complications / Q000378:metabolism*; D002779:Cholestasis / Q000150:complications / Q000378:metabolism; D004591:Electrophoresis, Polyacrylamide Gel; D005260:Female; D006801:Humans; D015151:Immunoblotting; D007223:Infant; D007231:Infant, Newborn; D008099:Liver / Q000378:metabolism; D008297:Male; D009752:Nutritional Status / Q000502:physiology*; D012177:Retinol-Binding Proteins / Q000378:metabolism*; D054839:Retinol-Binding Proteins, Plasma; D014801:Vitamin A / Q000097:blood / Q000627:therapeutic use*; D014802:Vitamin A Deficiency / Q000150:complications / Q000188:drug therapy / Q000378:metabolism*
chemical_list: D012177:Retinol-Binding Proteins; D054839:Retinol-Binding Proteins, Plasma; D014801:Vitamin A