Measurement of the TCA cycle rate in the dentate nucleus in Friedreichs Ataxia

OBJECTIVE: To measure the tricarboxylic acid (TCA) cycle rate in the dentate nucleus in a group of control subjects and subjects with Friedreich's Ataxia (FRDA). HYPOTHESIS: The TCA cycle rate will be lower in FRDA subjects than in controls APPROACH: We will infuse carbon-13 (13C) or deuterated (2H) labeled glucose and measure the rate of 13C label incorporation from glucose to glutamate in the brain using in vivo magnetic resonance spectroscopy.