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In the two crystal structures of normal human G6P, Pro172 is seen exclusively in the cis conformation, while in the crystal structure of one disease causing mutant (variant Canton R459L), Pro172 is seen almost exclusively in the trans conformation

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This equilibrium is particularly critical in high-energy-demand tissues, such as the heart, where copper serves as an indispensable cofactor for key metabolic enzymes and mitochondrial function [18, 19]

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in those preclinical studies

Accessed 03 March 2020