NAD+ and Exercise in FA

Exclusion Criteria

• •1. Known sensitivity to NR.
• •2. Concurrent use of any medications, including statins, likely to increase risk of NR toxicity.
• •3. HgbA1c \> 8.5% and/or Diabetes Mellitus (DM) requiring insulin or insulin secretagogue.
• •4. Use of supraphysiologic steroids.
• •5. Laboratory abnormalities that indicate clinically significant anemia or bleeding risk. (Hemoglobin \< 10 g/dL or Platelets \< 100K)
• •6. Laboratory abnormalities that indicate clinically significant kidney disease using serum creatinine and Modification of Diet in Renal Disease (MDRD) equation. (Estimated Glomerular Filtration Rate (eGFR) \< 60 ml/min/1.73 m2)
• •7. Laboratory abnormalities that indicate clinically significant liver disease. (Aspartate Aminotransferase (AST)/Serum Glutamic Oxaloacetic Transaminase (SGOT) 3.0 x Upper Limit of Normal and/or Alanine Aminotransferase (ALT)/Serum Glutamic Pyruvic Transaminase (SGPT) 3.0 x Upper Limit of Normal)
• •8. Uncontrolled and persistent arrhythmias that are felt to be clinically significant.
• •9. Known history of moderate or severe left ventricular systolic dysfunction (Left Ventricular Ejection Fraction (LVEF) \< 45%)
• •10. Standard contraindications to exercise testing.
• •11. Inability to sit and pedal unassisted in a cycle ergometry chair, at a cadence of at least 55 rotations per minute (rpm) during unloaded warm up, in a cycle ergometry chair and complete a maximal Cardio Pulmonary Exercise Test (CPET)
• •12. Inability to sit and pedal unassisted in a recumbent tricycle.
• •13. Any contraindication to MRI. Including:
• •* Any intra-luminal implant, filter, stent or valve replacement
• •* Any type of life assist device, pump, or prosthetic
• •* Any vascular clip or clamp
• •* Any surgically placed clips or clamps or bands on visceral organs
• •* Any intracranial implants of any type other than dental fillings
• •* Any non-removable piercings, jewelry, or medicinal patch
• •* Any personal history of intraocular injury or fragment in or around the orbit that cannot be cleared through radiologic examination.
• •* Any personal history of bullet, shrapnel, or stabbing wounds that cannot be cleared through radiologic evaluation.
• •* Inability to lie flat in the MRI scanner for 60-90 minutes.
• •14. Use of any investigational agent within 4 weeks of enrollment, except open-label extension phase.
• •15. Females: pregnant, lactating, or planning to become pregnant during their participation.
• •16. Any medical condition, in the opinion of the investigator that will interfere with the safe completion of the study.
• •17. Parents/guardians or participants who, in the opinion of the Investigator, may be non-compliant with study schedules or procedures.