Hahn S H, Tanner M S, Danks D M, Gahl W A
Section on Human Biochemical Genetics, National Institute of Child Health and Human Development, National Institutes of Health Bethesda, Maryland 20892, USA
Biochem Mol Med. 1995 Apr;54(2):142-5. doi: 10.1006/bmme.1995.1021.
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.