Wilson's disease, also known as hepatolenticular degeneration, is a genetic disease with a deficiency on regulation of copper in the human bodies. Besides the high level of copper in the body, the patients with Wilson's disease were detects in their livers significantly higher level of etheno adducts 1,N-ethenodeoxyadenosine and 3,N-ethenodeoxycytine, which are the substrates of the AlkB family DNA repair enzymes. The α-ketoglutarate (αKG)/Fe(II)-dependent AlkB family DNA repair enzymes is the major protein to oxidize those alkyl group and subsequently remove adducts from damaged bases. Recently, studies demonstrated that AlkB's human homolog, ABH2, can be inhibited by toxic metals like nickel ion via replacing the ferrous iron in the catalytic center. In vitro biochemical assay showed that Cu(II) ions could directly inhibit the activity of AlkB enzymes. This project is aiming to illustrate the relationship of AlkB's inhibition by copper with the accumulation of etheno adducts in Wilson's disease patients.

Copper inhibits AlkB family DNA repair enzymes and the relationship with Wilson's disease

251st National Meeting (2016)

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