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Phase 2 N=36 Randomized Quadruple-blind Treatment

Safety, Tolerability, and Efficacy of MTP-131 for the Treatment of Mitochondrial Myopathy

Mitochondrial Myopathy

Enrolled (actual)
36
Serious AEs
0.0%
Results posted
Dec 2019
Primary outcome: Primary: Change in Distance Walked (Meters) on the 6-minute Walk Test (6MWT) — 13.5; 36.5; 64.5; 20.4 meters

Study Design & Population

Study type
Interventional
Phase
Phase 2
Interventions
elamipretide (low dose) (Drug); elamipretide (intermediate dose) (Drug); elamipretide (high dose) (Drug); Placebo (Drug)
Age
Pediatric, Adult, Older Adult · 16+ yrs
Sex
All
Sponsor
Stealth BioTherapeutics Inc.
Primary completion
Apr 2016

Outcome Measures

OutcomeResultp-value
PRIMARY
Change in Distance Walked (Meters) on the 6-minute Walk Test (6MWT)
13.5; 36.5; 64.5; 20.4
SECONDARY
Change in Maximum Oxygen Uptake (ml/kg/Min)
0.35; 2.36; 0.64; 2.14
SECONDARY
Change in Ventilatory Efficiency (VE/VCO2 Slope)
0.749; -0.671; -1.681; -1.375
SECONDARY
Change in Aerobic Efficiency (ΔO2 Consumption/Δ Work Ratio)
-0.234; 0.643; -0.214; 0.738
SECONDARY
Change in Oxygen Utilization (ΔVO2/ΔlogVE Ratio)
-118.87; 184.99; 90.13; 166.50
SECONDARY
Change in Oxygen Uptake Kinetics (Mean Response Time as Measured by Seconds)
6.40; -2.49; -9.75; -7.47
SECONDARY
Change in Pre-exercise Lactate Levels (mg/dL)
3.4; 13.8; 0.4; 4.4
SECONDARY
Change in Post-exercise Lactate Levels (mg/dL)
6.9; 10.6; 4.3; 8.4
SECONDARY
Change in Peak Respiratory Exchange Ratio (VCO2/VO2)
0.109; 0.114; 0.010; 0.133
SECONDARY
Change in Peak Respiratory Rate (Breaths/Min)
1.4; -0.7; 0.9; 8.2
SECONDARY
Change in Peak Ventilation (L/Min)
2.70; 8.87; 2.49; 8.47
SECONDARY
Change in Peak Heart Rate (Beats/Min)
10.1; 16.7; 4.5; 3.8
SECONDARY
Change in Peak Oxygen Saturation (% O2-saturated Hemoglobin)
0.0; 0.1; 0.6; 0.3
SECONDARY
Change in Peak Systolic Blood Pressure (mmHg)
16.7; -1.5; 16.0; -2.7
SECONDARY
Change in Peak Diastolic Blood Pressure (mmHg)
0.6; -1.4; -2.5; -6.6
SECONDARY
Change in Peak Borg Dyspnea
-2.00; -2.2; -1.4; -0.4
SECONDARY
Change in VO2 Anaerobic Threshold (mL)
30.00; 10.8; 1.6; 42.0
SECONDARY
Change in Watts
9.6; 13.9; 5.4; 4.4
SECONDARY
Change in Temperature (°C)
-0.3; 0.2; 0.1; 0.0
SECONDARY
Change in ECG-PR Interval (Msec)
-3.2; 8.3; 9.1; -1.6
SECONDARY
Change in ECG-QRS Complex (Msec)
1.2; 2.3; -2.2; 0.8
SECONDARY
Change in ECG-QT Interval (Msec)
-6.0; -4.8; -16.7; -1.3
SECONDARY
Change in ECG-QTc Interval (Msec)
3.4; 1.3; -23.2; 6.7
SECONDARY
Number of Participants Who Had Suicide Ideation, Suicidal Behavior, or Non-suicidal Self-injurious Behavior Post-screening.
0; 0; 0; 0
SECONDARY
Change in Creatine Phosphokinase (IU/L)
-24.8; -161.7; -101.2; -154.9
SECONDARY
Change in Alanine Aminotransferase (ALT) (U/L)
19.3; -3.0; -5.3; 1.1
SECONDARY
Change in Aspartate Aminotransferase (AST) (U/L)
5.6; -2.2; -12.6; -4.4
SECONDARY
Change in Eosinophils (10^9 Cells/L)
0.06; 0.08; -0.01; 0.04

Summary

Phase 1/2, multi-center, randomized, double-blind, multiple ascending dose, placebo-controlled study that enrolled 36 subjects with mitochondrial myopathy associated with genetically confirmed mitochondrial disease to evaluate the safety, tolerability, pharmacokinetics (PK), and preliminary efficacy of MTP-131 in this patient population.

Eligibility Criteria

Inclusion Criteria

  • Diagnosis of mitochondrial disease believed to impair the mitochondrial respiratory chain.
  • Eligibility requires prior genetic confirmation of mitochondrial disease.
  • Diagnosis of mitochondrial myopathy judged by the Investigators to be due to existing mitochondrial disease.
  • Must be able to complete a Screening Visit 6MWT.
  • Body mass index (BMI) score >15.0 and 450 msec in male subjects and >480 msec in female subjects.
  • Uncontrolled hypertension (>160 mmHg systolic or >100 mmHg diastolic) at Screening Visit.
  • History of rhabdomyolysis defined as an acute rise in the serum creatine phosphokinase (CPK) value that, in the opinion of the investigator, caused clinically significant symptoms.
  • Serum sodium more than 5 meq/L below the reference lower limit of normal at Screening Visit.
  • Participated in another interventional clinical trial within 3 months of the screening visit or is currently enrolled in a non-interventional clinical trial judged by the Investigator to be incompatible with the current trial.
  • Other protocol-defined inclusion/exclusion criteria may apply.
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT02367014). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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