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This Concept Map, created with IHMC CmapTools, has information related to: PDC, gluconeogenesis produces glucose when insulin/glucagon is low, Mother 26yrs old gives birth to Elizabeth, ADP inhibit Pyruvate dehydrogenase kinase (PDK), variability in disease presentation contingent on three factors X chromosome lyonization, Pyruvate dehydrogenase (E1) consists of E1β subunit, Pyruvate Dehydrogenase Deficiency requires Disease management/ therapy, Pyruvate inhibit Pyruvate dehydrogenase kinase (PDK), Disease management/ therapy includes Thiamine, thiamine pyrophosphate (TPP) is a cofactor for Pyruvate dehydrogenase (E1), ketone bodies can be relied on during fasting or in PDC deficiency for energy production CNS, Anabolic pathways specifically gluconeogenesis, NADH production leads to increased NADH/NAD+ ratio, Pyruvate Dehydrogenase Complex (PDC) can also divert substrates toward Anabolic pathways, Elizabeth presents with E1 alpha protein detected by Western blot, Elizabeth has normal levels of Blood glucose, Pyruvate Dehydrogenase Deficiency specifically in the E1α subunit, Dihydrolipoyl dehydrogenase (E3) have Normal Residual activity, ketosis is an elevated level of ketone bodies, Pyruvate Dehydrogenase Phosphatase (PDP) is stimulated by Magnesium, X chromosome demonstrating X-linked dominant inheritance