Low molecular weight copper binding proteins in Wilson disease.

Cultured fibroblasts deriving from Wilson disease patients were compared with the control ones in respect of copper accumulation and low molecular weight copper binding protein (metallothionein) properties. No evidence was obtained that metallothionein abnormality could be a primary cause of copper metabolism disturbances in Wilson disease. The determination of radioactivity, present in serum low molecular weight fraction 24 hours after intravenous injection of 64Cu, has been suggested as an additional tool in Wilson disease diagnosis in doubtful cases.