Clinical Trial of Coenzyme Q10 and Lisinopril in Muscular Dystrophies

Inclusion Criteria:

- 8 years of age or older

- Confirmed genetic diagnosis of Duchenne, Becker, or Limb Girdle muscular dystrophy

- Beta-blocker naïve

- Screening Doppler echocardiographic MPI measurement greater than or equal to 0.40 for
the highest MPI value (spectral and tissue) or circumferential strain measured by STE
that is less negative than or equal to - 23

- Normal left ventricular fractional shortening (≥28%) and no clinical cardiac symptoms

- Has not participated in other therapeutic research protocol within the last 6 months
prior to screening

- Ability to swallow tablets

Exclusion Criteria:

- Spine curvature greater than 30% (based on the x-ray performed at screening)

- History of significant concomitant illness or significant impairment of renal or
hepatic function

- History of hypersensitivity to ACE inhibitors

- History of idiopathic or hereditary angioedema or a history of angioedema with prior
ACE inhibitor use

- Use of carnitine, creatine, glutamine, or any herbal medicines (this would not include
herbal teas unless they are consumed daily with intended medicinal effect) in the
3-months prior to enrollment

- CoQ10 and/or ACE inhibitor use for a duration greater than 6 months

- CoQ10 and/or ACE inhibitor use in the 3-months prior to enrollment

- CoQ10 serum level of 2.5 ug/ml or higher

- Investigator assessment of inability to comply with protocol