Functional analysis and drug response to zinc and D-penicillamine in stable ATP7B mutant hepatic cell lines

AIM: To study the effect of anti-copper treatment for survival of hepatic cells expressing different ATP7 B mutations in cell culture. METHODS: The most common Wilson disease(WD) mutations p.H1069 Q, p.R778 L and p.C271*, found in the ATP7 B gene encoding a liver copper transporter, were studied. Th...

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Veröffentlicht in:	World journal of gastroenterology : WJG 2016-04, Vol.22 (16), p.4109-4119
Hauptverfasser:	Chandhok, Gursimran, Horvath, Judit, Aggarwal, Annu, Bhatt, Mohit, Zibert, Andree, Schmidt, Hartmut Hj
Format:	Artikel
Sprache:	eng
Schlagworte:	Adenosine Triphosphatases - genetics Adenosine Triphosphatases - metabolism Apoptosis - drug effects ATP7B D-penicillamine Zinc Mutations Wilson Basic Study Carcinoma, Hepatocellular - enzymology Carcinoma, Hepatocellular - genetics Carcinoma, Hepatocellular - pathology Cation Transport Proteins - genetics Cation Transport Proteins - metabolism Cell Survival - drug effects Chelating Agents - pharmacology Chlorides - pharmacology Copper - toxicity Copper-transporting ATPases disease Therapy Dose-Response Relationship, Drug Genotype Hep G2 Cells Hepatocytes - drug effects Hepatocytes - enzymology Hepatocytes - pathology Hepatolenticular Degeneration - drug therapy Hepatolenticular Degeneration - enzymology Hepatolenticular Degeneration - genetics Hepatolenticular Degeneration - pathology Humans Liver Neoplasms - enzymology Liver Neoplasms - genetics Liver Neoplasms - pathology Mutation Penicillamine - pharmacology Phenotype Protein Transport Transfection Zinc Compounds - pharmacology
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