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glucose 1-phosphate cori ester anomeric catabolic glycogenolysis glycogen phosphorylase molecule glucose glycogen storage disease type mcardle disease enzyme phosphoglucomutase cell membrane catabolism gsd xiv udp-glucose synthase maltose kojibiose trehalose rendering metabolite metabolism biosynthesis cycle mechanism pathway path biosynthetic intermediate biogenesis substrate precursor synthesis derivative bioprecursor metabolic enzyme endogenous contractor human metabolome database hmdb