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This Concept Map, created with IHMC CmapTools, has information related to: Biochem A1 PDC Deficiency, Elizabeth diagnostic tests show 5.7 mmol/L blood lactate (fed state), NADH/NAD+ ratio in cytosol, delayed development shown by delayed developmental milestones, Acetyl CoA activates Pyruvate Dehyrdogenase Kinase, TCA cycle providing NADH, Ann placed on ketogenic diet, pyruvate activating lactate dehydrogenase, Elizabeth presents with floppiness, Mother 26 y.o. gave birth to Elizabeth, ATP deficit disproportionately affects Tissues, ADP inactivates Pyruvate Dehyrdogenase Kinase, pyruvate to lactate, delayed development shown by floppiness, Elizabeth diagnostic tests show 5.6 mmol/L in CSF (fasting), Acetyl CoA leading to increase of NADH/NAD+ ratio, Acetyl CoA decreases activity of TCA cycle, recurrent upper respiratory illnesses less severe in Ann, Pyruvate Dehydrogenase Complex to enter TCA cycle, Ann as compared to Elizabeth, Tissues which have High Energy Requirements