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This Concept Map, created with IHMC CmapTools, has information related to: Biochem A1 MELAS, 17 yr old Female Caucasian presents with vomiting, carnitine induces fatty acid oxidation, Mitochondrial inheritance of mtDNA, <math xmlns="http://www.w3.org/1998/Math/MathML"> <mrow> <mtext> hydrogen peroxide ( </mtext> <mmultiscripts> <mtext> H </mtext> <mtext> 2 </mtext> <none/> </mmultiscripts> <mmultiscripts> <mtext> O </mtext> <mtext> 2 </mtext> <none/> </mmultiscripts> <mtext> ) </mtext> </mrow> </math> which are reactive oxygen species (ROS), cell death of Brain cells, cardiac conduction defects is a Clinical manifestation, complex II (succinate DH) passes electron to CoQ, Energy used to Pump H+, Pyruvate which showed high lactate:pyruvate ratio, Idebenon protects inner membrane, complex II (succinate DH) provides an alternative route to continue Electron Transport Chain, high error rate increasing presence of mtDNA mutation, mt matrix to the inner membrane, anaerobic glycolysis which produces Pyruvate, intracellular creatine/phosphocreatine level is needed to maintain sufficient cellular ATP level, leaking of antioxdiant fails to neutralize reactive oxygen species (ROS), lipid damage causes cellular damage, 17 yr old Female Caucasian presents with muscle weakness, Pump H+ from the mt matrix, complex III (cytochrome bc1 complex) pases electron to complex IV (cytochrome a+a3)