title: Normal metallothionein synthesis in fibroblasts obtained from children with Indian childhood cirrhosis or copper-associated childhood cirrhosis.
abstract: We previously demonstrated decreased metallothionein (MT) synthesis in cultured fibroblasts obtained from an American boy with findings typical of Indian Childhood Cirrhosis (ICC). We now report normal basal, copper-induced, and zinc-induced MT synthesis in the fibroblasts of two Indian boys and one Irish boy with typical ICC and one Indian boy with copper-associated childhood cirrhosis. This suggests that etiologies other than impaired MT production should be sought as the primary defect in these disorders.
authors.affiliation: Section on Human Biochemical Genetics, National Institute of Child Health and Human Development, National Institutes of Health Bethesda, Maryland 20892, USA
authors.forename: S H
authors.initials: SH
authors.lastname: Hahn
authors.forename: M S
authors.initials: MS
authors.lastname: Tanner
authors.forename: D M
authors.initials: DM
authors.lastname: Danks
authors.forename: W A
authors.initials: WA
authors.lastname: Gahl
mesh_terms: D001345:Autoradiography; D002478:Cells, Cultured; D002648:Child; D002675:Child, Preschool; D003300:Copper / Q000009:adverse effects* / Q000378:metabolism / Q000494:pharmacology; D003545:Cysteine / Q000737:chemistry; D004591:Electrophoresis, Polyacrylamide Gel; D005260:Female; D005347:Fibroblasts / Q000166:cytology / Q000187:drug effects / Q000378:metabolism*; D006801:Humans; D007194:India; D007223:Infant; D008103:Liver Cirrhosis / Q000139:chemically induced / Q000188:drug therapy / Q000378:metabolism*; D008297:Male; D008668:Metallothionein / Q000096:biosynthesis* / Q000187:drug effects; D013462:Sulfur Radioisotopes; D015032:Zinc / Q000494:pharmacology
chemical_list: D013462:Sulfur Radioisotopes; D003300:Copper; D008668:Metallothionein; D015032:Zinc; D003545:Cysteine