Normal metallothionein synthesis in fibroblasts obtained from children with Indian childhood cirrhosis or copper-associated childhood cirrhosis

S H Hahn; M S Tanner; D M Danks; W A Gahl

doi:10.1006/bmme.1995.1021

Normal metallothionein synthesis in fibroblasts obtained from children with Indian childhood cirrhosis or copper-associated childhood cirrhosis

Biochem Mol Med. 1995 Apr;54(2):142-5. doi: 10.1006/bmme.1995.1021.

Authors

S H Hahn¹, M S Tanner, D M Danks, W A Gahl

Affiliation

¹ Section on Human Biochemical Genetics, National Institute of Child Health and Human Development, National Institutes of Health Bethesda, Maryland 20892, USA

PMID: 8581360
DOI: 10.1006/bmme.1995.1021

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.

MeSH terms

Autoradiography
Cells, Cultured
Child
Child, Preschool
Copper / adverse effects*
Copper / metabolism
Copper / pharmacology
Cysteine / chemistry
Electrophoresis, Polyacrylamide Gel
Female
Fibroblasts / cytology
Fibroblasts / drug effects
Fibroblasts / metabolism*
Humans
Indien
Infant
Liver Cirrhosis / chemically induced
Liver Cirrhosis / drug therapy
Liver Cirrhosis / metabolism*
Male
Metallothionein / biosynthesis*
Metallothionein / drug effects
Sulfur Radioisotopes
Zinc / pharmacology

Substances

Sulfur Radioisotopes
Copper
Metallothionein
Zinc
Cysteine