DIFFERENCE IN STABILITY OF THE N-DOMAIN UNDERLIES DISTINCT INTRACELLULAR PROPERTIES OF THE E1064A AND H1069Q MUTANTS OF Cu- TRANSPORTING ATPASE ATP7B
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چکیده
DIFFERENCE IN STABILITY OF THE N-DOMAIN UNDERLIES DISTINCT INTRACELLULAR PROPERTIES OF THE E1064A AND H1069Q MUTANTS OF CuTRANSPORTING ATPASE ATP7B. Oleg Y. Dmitriev, Ashima Bhattacharjee, Sergiy Nokhrin, Eva-Maria E. Uhlemann, and Svetlana Lutsenko Department of Biochemistry, University of Saskatchewan, Saskatchewan, S7N 5E5, Canada Department of Physiology, Johns Hopkins University, Baltimore, MD USA Running head: Structure and Stability of Wilson disease mutant E1064A Address correspondence to: Oleg Dmitriev, 107 Wiggins Rd., Saskatoon, SK S7N 5E5, Canada. Tel: 306966-4377 Fax: 306-966-4390, E-mail: [email protected] and Svetlana Lutsenko 725N Wolfe St., Baltimore MD, 21205, Tel:410-614-4661, [email protected]
منابع مشابه
Difference in stability of the N-domain underlies distinct intracellular properties of the E1064A and H1069Q mutants of copper-transporting ATPase ATP7B.
Wilson disease (WD) is a disorder of copper metabolism caused by mutations in the Cu-transporting ATPase ATP7B. WD is characterized by significant phenotypic variability, the molecular basis of which is poorly understood. The E1064A mutation in the N-domain of ATP7B was previously shown to disrupt ATP binding. We have now determined, by NMR, the structure of the N-domain containing this mutatio...
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تاریخ انتشار 2011