Normal metallothionein synthesis in fibroblasts obtained from children with Indian childhood cirrhosis or copper-associated childhood cirrhosis.
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.
Hahn
S H
SH
Section on Human Biochemical Genetics, National Institute of Child Health and Human Development, National Institutes of Health Bethesda, Maryland 20892, USA
Tanner
M S
MS
Danks
D M
DM
Gahl
W A
WA
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
D013462:Sulfur Radioisotopes; D003300:Copper; D008668:Metallothionein; D015032:Zinc; D003545:Cysteine