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Hepatolenticular Degeneration

Data on hepatolenticular degeneration detailed by R. Pilankatta and co-authors

Published in Virus Weekly, October 6th, 2009

"ATP7B is a P-type ATPase required for copper homeostasis and related to Wilson disease of humans. In addition to various domains corresponding to other P-type ATPases, ATP7B includes an N terminus extension (NMBD) with six copper binding sites," researchers in the United States report.

"We obtained high yield expression of WT and mutant ATP7B in COS1 cells infected with adenovirus vector. ATP7B, isolated with the microsomal fraction of cell homogenates, accounts for 10-20% of the total protein. Copper-dependent, steady-state ATPase yields 30 nmol of Pi/mg of protein/min at 37 degrees C, pH 6.0. ATP7B phosphorylation with ATP occurs with diphasic kinetics and is totally...

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