Laser ablation inductively coupled plasma mass spectrometry imaging of metals in experimental and clinical Wilson's disease.

ABSTRACT: Wilson's disease is an autosomal recessive disorder in which the liver does not properly release copper into bile, resulting in prominent copper accumulation in various tissues. Affected patients suffer from hepatic disorders and severe neurological defects. Experimental studies in mutant mice in which the copper-transporting ATPase gene (Atp7b) is disrupted revealed a drastic, time-dependent accumulation of hepatic copper that is accompanied by formation of regenerative nodes resembling cirrhosis. Therefore, these mice represent an excellent exploratory model for Wilson's disease. However, the precise time course in hepatic copper accumulation and its impact on other trace metals within the liver is yet poorly understood. We have recently established novel laser ablation inductively coupl

SUBMITTER: Boaru SG 

PROVIDER: S-EPMC4395195 | biostudies-literature | 2015 Apr

REPOSITORIES: biostudies-literature