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Energy expenditure, substrate utilization, food intake, and spontaneous activity were determined 45,47 , based upon O 2 consumption and CO 2 production, using a Mouse CLAMS System (Columbus Instruments, Columbus, OH)

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IEM are a class of inborn errors of metabolic pathways that are marked by accumulation (and usually toxic) organic acid metabolites in blood (i.e., organic acidemias) and increased excretion of organic acids in urine (i.e., organic acidurias)

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