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Omaveloxolone

Phase 3

Friedreich Ataxia | Small molecule | Rare Disease |Biogen Inc.|Last Updated: Sep 3, 2026

Target and mechanism

Molecular targetNFE2L2
Target classActivator
ModalitySmall molecule

Success Probability

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Market & Valuation

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Trial Design

RandomizedDouble-BlindCONTROLLEDDMC
Total Trials3
Total Enrollment460

FDA Designations

No designations recorded

Clinical trial landscape

Omaveloxolone · 16 trials · 13 indications

Phase 3 1Phase 2 2Phase 1 13
NCT06953583A Study to Learn More About the Effects and Long-Term Safety of Omaveloxolone (BIIB141) in Children and Teens With Friedreich's AtaxiaFriedreich Ataxia
RECRUITING255 Analytics
PHASE3RECRUITING
A Study to Learn More About the Effects and Long-Term Safety of Omaveloxolone (BIIB141) in Children and Teens With Friedreich's Ataxia
Friedreich AtaxiaUnlock trial analytics

Study Endpoints

Primary Endpoints

Part 1: Change From Baseline in Upright Stability Score (USS) Subscale E of Modified Friedreich's Ataxia Rating Scale (mFARS) at Week 52
Baseline, Week 52

The mFARS is a validated and sensitive rating scale that was developed to quantitatively assess the severity of the neurologic features of FA in adults and adolescents. Scores on the mFARS range from 0 to 93, with lower scores indicating better neurological function. The subscales of the mFARS assessment and maximum score for each subscale are: bulbar function (Subscale A; 2 assessments of speech and cough; maximum score = 5), upper limb coordination (Subscale B; 5 assessments of coordination of movement and function in arms and hands with each limb scored individually; maximum score = 36), lower limb coordination (Subscale C; 2 assessments of coordination of movement and function of lower limbs with each limb scored individually; maximum score = 16), and upright stability (USS, Subscale E; 9 assessments of sitting posture, stance, tandem walk, and gait assessments; maximum score = 36).

Part 2A: Change From Baseline in USS Subscale E of mFARS at Week 52
Baseline (Week 52 of Part 1), Week 52

The mFARS is a validated and sensitive rating scale that was developed to quantitatively assess the severity of the neurologic features of FA in adults and adolescents. Scores on the mFARS range from 0 to 93, with lower scores indicating better neurological function. The subscales of the mFARS assessment and maximum score for each subscale are: bulbar function (Subscale A; 2 assessments of speech and cough; maximum score = 5), upper limb coordination (Subscale B; 5 assessments of coordination of movement and function in arms and hands with each limb scored individually; maximum score = 36), lower limb coordination (Subscale C; 2 assessments of coordination of movement and function of lower limbs with each limb scored individually; maximum score = 16), and upright stability (USS, Subscale E; 9 assessments of sitting posture, stance, tandem walk, and gait assessments; maximum score = 36).

Part 2B: Number of Participants With Treatment-Emergent Adverse Event (TEAE) and Treatment-Emergent Serious Adverse Event (TESAE)
From the first dose of the study drug in Part 2B up to the end of follow-up period in Part 2B (up to Week 104)
Part 2B: Number of Participants With Change From Baseline in Cardiac Function Assessed by Echocardiogram (ECHO) at Weeks 52 and Week 104
Baseline (Week 52 of Part 1), Weeks 52 and 104
Part 2B: Change From Baseline in Height at Weeks 52 and Week 104
Baseline (Week 52 of Part 1), Weeks 52 and 104
Part 2B: Change From Baseline in Weight at Weeks 52 and Week 104
Baseline (Week 52 of Part 1), Weeks 52 and 104
Part 2B: Change From Baseline in Body Mass Index (BMI) at Weeks 52 and Week 104
Baseline (Week 52 of Part 1), Weeks 52 and 104
Part 2B: Change From Baseline in Columbia Suicide Severity Rating Scale (C-SSRS) at Weeks 52 and Week 104
Baseline (Week 52 of Part 1), Weeks 52 and 104

The C-SSRS is a low-burden measure of the spectrum of suicidal ideation and behavior that was developed to assess severity and track suicidal events through any treatment of individuals ≥ 6 years of age. The C-SSRS is a clinical interview providing a summary of both ideation and behavior that can be administered by the clinician during any evaluation or risk assessment to identify the level and type of suicidality present. The assessment includes "yes" or "no" responses for 5 questions each, related to suicidal ideation (wish to be dead, non-specific active suicidal thoughts, active suicidal ideation with any methods, active suicidal ideation with some intent, active suicidal ideation with specific plan) and suicidal behavior (preparatory acts or behavior, aborted attempt, interrupted attempt, actual attempt, suicide). Numeric ratings are provided for severity of ideation, from 1 to 5, with 5 being the most severe.

Part 2B: Percentage of Participants at Each Tanner Stage at Weeks 52 and Week 104
Baseline (Week 52 of Part 1), Weeks 52 and 104

Assessment of Tanner stages (a scale of physical development) will be performed by a medical doctor experienced with this assessment. Tanner score ranges from Stage 1 (childhood) to Stage 5 (full physical maturity). Information regarding Tanner staging will be collected at baseline for all participants and will be stopped once the participant reaches Tanner Stage 5 in all gender-appropriate scales.

Part 2B: Number of Participants at Each Tanner Stage at Weeks 52 and Week 104
Baseline (Week 52 of Part 1), Weeks 52 and 104

Assessment of Tanner stages (a scale of physical development) will be performed by a medical doctor experienced with this assessment. Tanner score ranges from Stage 1 (childhood) to Stage 5 (full physical maturity). Information regarding Tanner staging will be collected at baseline for all participants and will be stopped once the participant reaches Tanner Stage 5 in all gender-appropriate scales.

Change of Peak Workload (in Watts/kg) During Exercise Testing
12 weeks

Cycle ergometry using a stationary recumbent bike was used to conduct maximal exercise testing. Peak work is defined as the workload at which patients reach maximal volition (defined as an inability to continue to exercise due to exhaustion). Change of peak workload during exercise testing was measured at baseline, Week 4, and Week 12. Change from baseline at Week 12 reported.

Part 1: Change From Baseline in Peak Work During Maximal Exercise Testing at Week 12
Baseline, Week 12

Cycle ergometry using a recumbent stationary bicycle was used to conduct maximal exercise testing and workload was increased incrementally. Peak work is defined as the workload at which participants reach maximal volition (defined as an inability to continue to exercise due to exhaustion). A positive change from baseline suggests an improvement.

Part 1: Number of Participants With Treatment Emergent Adverse Events (TEAEs) and Treatment Emergent Serious Adverse Events (TESAEs)
From first dose of study drug up to end of Part 1 of the study (up to Week 16)

An adverse event (AE) was any unfavorable and unintended sign (including any clinically significant abnormal laboratory test result), symptom, or disease temporally associated with the use of the study drug whether or not it is considered to be study drug related. An SAE was any untoward medical occurrence that at any dose resulted in death, in the view of the Investigator, placed the participant at immediate risk of death, required inpatient hospitalization or prolongation of existing hospitalization, resulted in persistent or significant disability/incapacity, resulted in a birth defect. TEAEs were defined as any AEs, regardless of relationship to study drug, that had an onset or worsened in severity on or after the first dose of study drug.

Part 2: Change From Baseline in the Modified Friedreich's Ataxia Rating Scale (mFARS) at Week 48
Baseline, Week 48

The Friedreich Ataxia Rating Scale (FARS) is a neurological-exam-based rating scale with five sections: Bulbar (section A), Upper Limb Coordination (section B), Lower Limb Coordination (section C), Peripheral Nervous System (section D), and Upright Stability (section E). mFARS is the sum of sections A (score 0 to 11), B (score 0 to 36), C (score 0 to 16), and E (score 0 to 36). The minimum score is 0 and the maximum score is 99. A lower score indicates better neurological function.

Part 2: Number of Participants With TEAEs and TESAEs
From first dose of study drug up to end of Part 2 of the study (up to Week 52)

An AE was any unfavorable and unintended sign (including any clinically significant abnormal laboratory test result), symptom, or disease temporally associated with the use of the study drug whether or not it is considered to be study drug related. An SAE was any untoward medical occurrence that at any dose resulted in death, in the view of the Investigator, placed the participant at immediate risk of death, required inpatient hospitalization or prolongation of existing hospitalization, resulted in persistent or significant disability/incapacity, resulted in a birth defect. TEAEs were defined as any AEs, regardless of relationship to study drug, that had an onset or worsened in severity on or after the first dose of study drug.

Maximum Observed Plasma Concentration (Cmax) of Omaveloxolone
Pre-dose and at multiple timepoints post-dose up to Day 29
Area Under the Concentration-Time Curve From Time Zero Extrapolated to Infinity (AUC0-inf) of Omaveloxolone
Pre-dose and at multiple timepoints post-dose up to Day 29
Area Under the Plasma Concentration-Time Curve From Time Zero to Last Measurable Concentration (AUC0-t) of Omeprazole
Pre-dose and at multiple timepoints post-dose on Days 1, 2, 16 and 17
Area Under the Plasma Concentration-Time Curve From Time Zero Extrapolated to Infinity (AUC0-inf) of Omeprazole
Pre-dose and at multiple timepoints post-dose on Days 1, 2, 16 and 17
Area Under the Concentration-Time Curve From Time Zero to Time of the Last Measurable Concentration (AUC0-t) of Omaveloxolone
Pre-dose and at multiple time points post dose up to Day 29
Maximum Observed Concentration (Cmax) of Omaveloxolone in Breast Milk
Predose and at multiple timepoints postdose (up to Day 15)
Time to Achieve Cmax (Tmax) of Omaveloxolone in Breast Milk
Predose and at multiple timepoints postdose (up to Day 15)
Average Concentration Based on Area Under the Concentration-Time Curve (AUC [Cav]) of Omaveloxolone in Breast Milk
Predose and at multiple timepoints postdose (up to Day 15)
Area Under the Concentration Time Curve From Time Zero to the Time of Last Measurable Concentration (AUC0-tlast) of Omaveloxolone in Breast Milk
Predose and at multiple timepoints postdose (up to Day 15)
Time of the Last Measurable Concentration (Tlast) of Omaveloxolone in Breast milk
Predose and at multiple timepoints postdose (up to Day 15)
AUC Time Curve From Time Zero to Infinity (AUCinf) of Omaveloxolone in Breast Milk
Predose and at multiple timepoints postdose (up to Day 15)
Milk-to-Plasma Ratio (M/P) of Omaveloxolone
Predose and at multiple timepoints postdose (up to Day 15)
Cumulative Amount of Omaveloxolone Excreted in Breast Milk (Ae) Over 24 Hours (Ae0-24) Postdose
At multiple timepoints postdose (up to 24 hours)
Cumulative Amount of Omaveloxolone Excreted in Breast Milk (Ae) Over 96 Hours (Ae0-96) Postdose
At multiple timepoints postdose (up to 96 hours)
Fraction of Omaveloxolone Excreted in Breast Milk (Fe) Over 24 Hours (Fe0-24)
At multiple timepoints postdose (up to 24 hours)
Fraction of Omaveloxolone Excreted in Breast Milk (Fe) Over 96 Hours (Fe0-96)
At multiple timepoints postdose (up to 96 hours)
Part 1: Apparent Clearance (CL/F) of Omaveloxolone
Predose (0 hour), after dose 1 hour, 2 hours, 3 hours, 4 hours, 24 hours, and 96 hours
Part 1: Maximum Concentration (Cmax) of Omaveloxolone
Predose (0 hour), after dose 1 hour, 2 hours, 3 hours, 4 hours, 24 hours, and 96 hours
Part 1: Volume of Distribution (V/F) of Omaveloxolone
Predose (0 hour), after dose 1 hour, 2 hours, 3 hours, 4 hours, 24 hours, and 96 hours
Part 1: Area Under the Plasma Concentration-Time Curve From 0 Extrapolated to Infinity (AUC0-∞) of Omaveloxolone
Predose (0 hour), after dose 1 hour, 2 hours, 3 hours, 4 hours, 24 hours, and 96 hours
Part 1: Area Under the Plasma Concentration-Time Curve From 0 to tlast (AUC0-tlast) of Omaveloxolone
Predose (0 hour), after dose 1 hour, 2 hours, 3 hours, 4 hours, 24 hours, and 96 hours
Part 1: Area Under the Plasma Concentration-Time Curve From 0 to 24 Hours (AUC0-24) of Omaveloxolone
Predose (0 hour), after dose 1 hour, 2 hours, 3 hours, 4 hours, 24 hours, and 96 hours
Part 1: Individual Steady-State AUC0-24 (AUC0-24,ss) of Omaveloxolone
Predose (0 hour), after dose 1 hour, 2 hours, 3 hours, 4 hours, 24 hours, and 96 hours
Part 1: Individual Steady-State Cmax (Cmax,ss) of Omaveloxolone
Predose (0 hour), after dose 1 hour, 2 hours, 3 hours, 4 hours, 24 hours, and 96 hours
Part 1: Concentration at the end of a 24-Hour Dosing Interval (Ctrough,ss) of Omaveloxolone
Predose (0 hour), after dose 1 hour, 2 hours, 3 hours, 4 hours, 24 hours, and 96 hours
Part 2: Number of Participants With Adverse Events (AEs) and Serious Adverse Events (SAEs)
From Day 1 up to the end of study (up to Week 240)

An AE is any untoward medical occurrence in a patient or clinical investigation participant administered a pharmaceutical product and that does not necessarily have a causal relationship with this treatment. An AE can therefore be any unfavorable and unintended sign (including abnormal laboratory finding), symptom, or disease temporally associated with use of a medicinal (investigational) product, whether or not related to medicinal (investigational) product. SAE is any untoward medical occurrence that at any dose results in death, in the view of investigator, places the participant at immediate risk of death (life-threatening event), requires inpatient hospitalization or prolongation of existing hospitalization, results in persistent or significant disability/incapacity, results in congenital anomaly/birth defect or is medically important event.

Part 2: Number of Participants With Clinically Significant Abnormality in Clinical Laboratory Assessments
From Day 1 up to Week 240
Part 2: Number of Participants With Clinically Significant Abnormality in Vital Signs
From Day 1 up to Week 240

Vital signs, including blood pressure (BP), heart rate (HR), and oral body temperature, will be assessed.

Part 2: Number of Participants With Clinically Significant Abnormality in Electrocardiograms (ECGs)
From Day 1 up to Week 240
Part 2: Number of Participants With Change from Baseline in Echocardiogram (ECHO)
From Day 1 up to Week 240
Part 2: Number of Participants With Change from Baseline in Height
From Day 1 up to Week 240
Part 2: Number of Participants With Change from Baseline in Weight
From Day 1 up to Week 240
Part 2: Number of Participants With Change from Baseline Body Mass Index (BMI)
From Day 1 up to Week 240
Part 2: Number of Participants With Change from Baseline in Tanner Assessment
From Day 1 up to Week 240
Part 2: Number of Participants With Change from Baseline in Paediatric Growth (Height)
From Day 1 up to Week 240
Part 2: Number of Participants With Change from Baseline in Paediatric Growth (Weight)
From Day 1 up to Week 240
Change from baseline in placebo-corrected QTcF (∆∆QTcF)
At Day 1 of each period, which is 14 days for both Treatment A and Treatment B and 4 days for Treatment C

The relationship between ∆QTcF and omaveloxolone and its metabolites, M17 and M22 plasma concentrations will be investigated by a linear mixed effects modeling approach with ∆ QTcF as the dependent variable, time-matched concentration of omaveloxolone and its metabolites as a continuous covariate (ie, 0 for placebo), and centered baseline QTcF as an additional covariate, treatment (active = 1; or placebo = 0) and time as categorical factors, and a random intercept and slope per subject.

Maximum concentration (Cmax) of omaveloxolone
43 days

Blood samples to assess omaveloxolone PK will be collected predose and at the specified time points over a 336-hour period following each omaveloxolone dose for the duration of the study.

Area under the plasma concentration-time curve from 0 to tlast (AUC0-tlas) of omaveloxolone
43 days

Blood samples to assess omaveloxolone PK will be collected predose and at the specified time points over a 336-hour period following each omaveloxolone dose for the duration of the study.

Area under the plasma concentration-time curve from 0 extrapolated to infinity (AUC0-∞) of omaveloxolone
43 days

Blood samples to assess omaveloxolone PK will be collected predose and at the specified time points over a 336-hour period following each omaveloxolone dose for the duration of the study.

Maximum concentration (Cmax) of omaveloxolone in plasma
15 days

Pharmacokinetics will be assessed by blood sampling for omaveloxolone to determine maximum observed concentration (Cmax).

Area under the the plasma omaveloxolone concentration-time curve (AUC)
15 days

Pharmacokinetics will be assessed by blood sampling for omaveloxolone to determine area under the curve (AUC).

Part 1 - Maximum concentration (Cmax) of probe drugs co-administered with omaveloxolone (midazolam, repaglinide, metformin, rosuvastatin, and digoxin)
28 days

Pharmacokinetics will be assessed by blood sampling for midazolam, repaglinide, metformin, rosuvastatin, and digoxin to determine maximum observed concentration (Cmax).

Part 1 - Area under the plasma concentration-time curve of (AUC) for probe drugs co-administered with omaveloxolone (midazolam, repaglinide, metformin, rosuvastatin, and digoxin)
28 days

Pharmacokinetics will be assessed by blood sampling for midazolam, repaglinide, metformin, rosuvastatin, and digoxin to determine area under the curve (AUC).

Part 2 - Maximum concentration (Cmax) of omaveloxolone
23 days

Pharmacokinetics will be assessed by blood sampling for omaveloxolone to determine maximum observed concentration (Cmax).

Part 2 - Area under the omaveloxolone concentration-time curve (AUC)
23 days

Pharmacokinetics will be assessed by blood sampling for omaveloxolone to determine area under the curve (AUC).

Part 3 - Maximum concentration (Cmax) of omaveloxolone
23 days

Pharmacokinetics will be assessed by blood sampling for omaveloxolone to determine maximum observed concentration (Cmax).

Part 3 - Area under the omaveloxolone concentration-time curve (AUC)
28 days

Pharmacokinetics will be assessed by blood sampling for omaveloxolone to determine area under the curve (AUC).

Part 4 - Maximum concentration (Cmax) of omaveloxolone
23 days

Pharmacokinetics will be assessed by blood sampling for omaveloxolone to determine maximum observed concentration (Cmax).

Part 4 - Area under the omaveloxolone concentration-time curve (AUC)
28 days

Pharmacokinetics will be assessed by blood sampling for omaveloxolone to determine area under the curve (AUC).

Area under the omaveloxolone concentration-time curve (AUC)
22 days

Pharmacokinetics will be assessed by blood sampling for omaveloxolone to determine area under the curve (AUC).

Maximum concentration of total radioactivity in blood and plasma
22 days

Mass balance and metabolite profiles will be assessed by blood sampling for omaveloxolone to determine maximum concentration of total radioactivity

Area under the concentration-time curve total radioactivity in blood and plasma
22 days

Mass balance and metabolite profiles will be assessed by blood sampling for total radioactivity to determine area under the concentration-time curve.

Amount of radioactivity excreted in urine (Aeu)
22 days

Rates and routes of elimination will be assessed by urine sampling for radioactivity.

Amount of radioactivity excreted in feces (Aef)
22 days

Rates and routes of elimination will be assessed by sampling of feces for radioactivity.

Determine the effect of food on the pharmacokinetics of omaveloxolone in healthy adult subjects by measuring maximum observed concentration (Cmax)
20 days

Pharmacokinetics will be assessed by blood sampling for omaveloxolone to determine maximum observed concentration (Cmax).

Determine the effect of food on the pharmacokinetics of omaveloxolone in healthy adult subjects by measuring area under curve (AUC)
20 days

Pharmacokinetics will be assessed by blood sampling for omaveloxolone to determine area under curve (AUC).

Measure of Efficacy of the Phase 2 Dose of RTA 408 in Combination With Nivolumab Using Overall Response Rate (ORR; Complete Plus Partial Responses) According to RECIST Version 1.1 Criteria
From enrollment up to the time of disease progression, up to 172 weeks for participants receiving Omaveloxolone in combination with Ipilimumab and 173 weeks for participants receiving Omaveloxolone combination with Nivolumab

Best overall response rate (ORR) is defined as the proportion of patients with complete or partial tumor size reduction according to RECIST v1.1 criteria. Stable disease is not a component of ORR. Complete response: Disappearance of all target lesions. Any pathological lymph nodes (whether target or non-target) must have reduction in short axis to \<10 mm. Partial reduction: At least a 30% decrease in the sum of diameters of target lesions, taking as reference the baseline sum diameters. The first occurrence of a response is considered an unconfirmed response. A CR or PR which persists to the next tumor burden assessment is then considered a confirmed response. Confirmed plus unconfirmed best overall response are presented. A subject may be counted twice if best unconfirmed response and best confirmed response are different.

Dose Determination
1 year (28-day cycles, up to 12 cycles per patient)

To determine the recommended Phase 2 dose of RTA 408 following oral administration to patients with metastatic or incurable NSCLC or melanoma that is relapsed, refractory after standard of care therapy, or for which standard of care therapy is not appropriate.

Secondary Endpoints

Part 1: Change From Baseline in Friedreich's Ataxia-Health Index (FA-HI) at Week 52
Baseline, Week 52
Part 1: Change From Baseline in Modified Friedreich's Ataxia Rating Scale (mFARS) at Week 52
Baseline, Week 52
Part 1: Change From Baseline in Patient Global Impressions-Severity (PGI-S) at Week 52
Baseline, Week 52
Unlock Study Endpoints

Study Design & Arms

AllocationRANDOMIZED
MaskingQUADRUPLE
ModelPARALLEL
PurposeTREATMENT

Treatment Arms

ArmTypeDescription
Part 1: OmaveloxoloneEXPERIMENTALParticipants will receive a single oral dose of omaveloxolone once a day (QD) for up to 52 weeks in Part 1 of the study.
Part 1: PlaceboPLACEBO_COMPARATORParticipants will receive placebo, orally, QD for up to 52 weeks in Part 1 of the study.
Part 2A Continued Efficacy Evaluation: OmaveloxoloneEXPERIMENTALParticipants will receive a single oral dose of omaveloxolone, QD for up to 104 weeks in Part 2A of the study.
Part 2B Safety: OmaveloxoloneEXPERIMENTALParticipants will receive a single oral dose of open-label omaveloxolone, QD for up to 104 weeks in Part 2B of the study.
omaveloxolone Capsules 2.5 mg and 5 mgEXPERIMENTALomaveloxolone (RTA 408) Capsules, 2.5 mg taken orally once daily for 2 weeks, then 5 mg taken orally once daily for 10 weeks
omaveloxolone Capsules 10 mgEXPERIMENTALomaveloxolone (RTA 408) capsules, 10 mg taken orally once daily for 12 weeks
Placebo CapsulesPLACEBO_COMPARATORPlacebo capsules taken orally once daily for 12 weeks
omaveloxolone Capsules 20 mgEXPERIMENTALomaveloxolone (RTA 408) Capsules, 20 mg taken orally once daily for 12 weeks.
omaveloxolone Capsules 40 mgEXPERIMENTALomaveloxolone (RTA 408) Capsules, 40 mg taken orally once daily for 12 weeks.
omaveloxolone Capsules 80 mgEXPERIMENTALomaveloxolone (RTA 408) Capsules, 80 mg taken orally once daily for 12 weeks.
omaveloxolone Capsules 160 mgEXPERIMENTALomaveloxolone (RTA 408) Capsules, 160 mg taken orally once daily for 12 weeks.
Part 1: Omaveloxolone 2.5 and 5 mgEXPERIMENTALParticipants received omaveloxolone 2.5 mg, oral capsule, QD for 2 weeks, followed by 5 mg Omaveloxolone, oral capsule, QD for 10 weeks.
Part 1: Omaveloxolone 10 mgEXPERIMENTALParticipants received omaveloxolone 10 mg, oral capsule, QD for 12 weeks.
Part 1: Omaveloxolone 20 mgEXPERIMENTALParticipants received omaveloxolone 20 mg, oral capsule, QD for 12 weeks.
Part 1: Omaveloxolone 40 mgEXPERIMENTALParticipants received omaveloxolone 40 mg, oral capsule, QD for 12 weeks.
Part 1: Omaveloxolone 80 mgEXPERIMENTALParticipants received omaveloxolone 80 mg, oral capsule, QD for 12 weeks.
Part 1: Omaveloxolone 160 mgEXPERIMENTALParticipants received omaveloxolone 160 mg, oral capsule, QD for 12 weeks.
Part 1: Omaveloxolone 300 mgEXPERIMENTALParticipants received omaveloxolone 300 mg, oral capsule, QD for 12 weeks.
Part 2: Omaveloxolone 150 mgEXPERIMENTALParticipants received omaveloxolone 150 mg, oral capsule, QD for 48 weeks.
Part 2: PlaceboPLACEBO_COMPARATORParticipants received placebo, oral capsule, QD for 48 weeks.
OLE: Placebo/ OmaveloxoloneEXPERIMENTALAll eligible participants who had received either omaveloxone or placebo in Part 1 and eligible participants who received placebo in Part 2 received omaveloxone, 150 mg, oral capsule, QD, until the drug was available through commercial channels or alternate post-trial access mechanisms, or until participant withdrawal, whichever was sooner (up to approximately 370 weeks).
OLE: Omaveloxolone/ OmaveloxoloneEXPERIMENTALAll eligible participants who had received omaveloxone in Part 2 continued to receive omaveloxolone, 150 mg, oral capsule, QD until the drug was available through commercial channels or alternate post-trial access mechanisms, or until participant withdrawal, whichever was sooner (up to approximately 370 weeks).
Treatment Sequence ABEXPERIMENTALParticipants will receive treatment A (omaveloxolone capsule orally) on Day 1 followed by treatment B (omaveloxolone TOS) on Day 15.
Treatment Sequence BAEXPERIMENTALParticipants will receive treatment B (omaveloxolone TOS) on Day 1 followed by treatment A (omaveloxolone capsule orally) on Day 15.
Period 1: OmeprazoleEXPERIMENTALParticipants will receive a single oral dose of omeprazole on Day 1.
Period 2: Omaveloxolone + OmeprazoleEXPERIMENTALParticipants will receive omaveloxolone from Days 2 to 15, followed by both omaveloxolone and omeprazole on Day 16.
OmaveloxoloneEXPERIMENTALParticipants will receive a single oral dose of omaveloxolone on Day 1.
Part 1 and 2: Cohort A1EXPERIMENTALCohort A1 will contain participants 12 to \<16 years of age. Participants will receive a single oral dose of omaveloxolone, 150 milligrams (mg), capsule, on Day 1 of the treatment period of part 1, followed by the same dose in part 2 up to when the last participant enrolled has the opportunity for their week 240. The dose in part 2 may be adjusted as per additional safety and Bayesian population pharmacokinetics (popPK) analyses.
Part 1 and 2: Cohort A2EXPERIMENTALCohort A2 will contain participants 12 to \<16 years of age. Participants will receive a single oral dose of omaveloxolone, capsule, at a dosage level determined by a Bayesian popPK analysis using the data from Cohort A1 to select the dose in part 1, followed by the same dose in part 2 up to when the last participant enrolled has the opportunity for their week 240. The dose in part 2 may be adjusted as per additional safety and Bayesian popPK analyses.
Part 1 and 2: Cohort B1EXPERIMENTALCohort B1 will contain participants 7 to \<12 years of age and will initiate in parallel with Cohort A2. Participants will receive a single oral dose of omaveloxolone, capsule, at a dosage level determined by a Bayesian popPK analysis using the data from Cohort A1 to select the dose in part 1, followed by the same dose in part 2 up to when the last participant enrolled has the opportunity for their week 240. The dose in part 2 may be adjusted as per additional safety and Bayesian popPK analyses.
Part 1 and 2: Cohort C1EXPERIMENTALCohort C1 will contain participants 2 to \<7 years of age. Participants will receive a single oral dose of omaveloxolone, capsule, at a dosage level determined by a Bayesian popPK analysis using the data from cohorts A1, A2, and B1 to select the dose in part 1, followed by the same dose in part 2 up to when the last participant enrolled has the opportunity for their week 240. The dose in part 2 may be adjusted as per additional safety and Bayesian popPK analyses.
Part 1 and 2: Cohort A3EXPERIMENTALCohort A3 will contain participants 12 to \<16 years of age. Participants will receive a single oral dose of omaveloxolone, capsule, at a dosage level determined by a Bayesian popPK analysis using the data from cohorts A1, A2, and B1 to select the dose in part 1, followed by the same dose in part 2 up to when the last participant enrolled has the opportunity for their week 240. The dose in part 2 may be adjusted as per additional safety and Bayesian popPK analyses.
Part 1 and 2: Cohort B2EXPERIMENTALCohort B2 will contain participants 7 to \<12 years of age and will initiate in parallel with Cohort A3. Participants will receive a single oral dose of omaveloxolone, capsule, at a dosage level determined by a Bayesian popPK analysis using the data from cohorts A1, A2, and B1 to select the dose in part 1, followed by the same dose in part 2 up to when the last participant enrolled has the opportunity for their week 240. The dose in part 2 may be adjusted as per additional safety and Bayesian popPK analyses.
Part 1 and 2: Cohort C2EXPERIMENTALCohort C2 will contain participants 2 to \<7 years of age. Participants will receive a single oral dose of omaveloxolone, capsule, at a dosage level determined by a Bayesian popPK analysis using the data from Cohorts A1, A2, A3, B1, B2, and C1 to select the dose in part 1, followed by the same dose in part 2 up to when the last participant enrolled has the opportunity for their week 240. The dose in part 2 may be adjusted as per additional safety and Bayesian popPK analyses.
Sequence ABCEXPERIMENTALSubjects receive three treatments (A: omaveloxolone 450 mg, B: omaveloxolone matching placebo 450 mg and C: moxifloxacin 400 mg) administered in a single dose with the standard FDA high-fat meal in three periods. Treatment A (Period 1 from Day 1 - 15), Treatment B (Period 2 from Day 15 - 29), and Treatment C(Period 3 from Day 29 -32)
Sequence BACEXPERIMENTALSubjects receive three treatments (A: omaveloxolone 450 mg, B: omaveloxolone matching placebo 450 mg and C: moxifloxacin 400 mg) administered in a single dose with the standard FDA high-fat meal in three periods. Treatment B (Period 1 from Day 1-15), Treatment A (Period 2 from Day 15 - 29, and Treatment C (Period 3 from Day 29 to 32)
Sequence ACBEXPERIMENTALSubjects receive three treatments (A: omaveloxolone 450 mg, B: omaveloxolone matching placebo 450 mg and C: moxifloxacin 400 mg) administered in a single dose with the standard FDA high-fat meal in three periods. Treatment A (Period 1 from Day 1-15), Treatment C (Period 2 from Day 15 -18), and Treatment B (Period 3from Day 18 - 32)
Sequence BCAEXPERIMENTALSubjects receive three treatments (A: omaveloxolone 450 mg, B: omaveloxolone matching placebo and C: moxifloxacin 400 mg) administered in a single dose with the standard FDA high-fat meal in three periods. Treatment B (Period 1 from Day 1 - 15), Treatment C (Period 2 from Day 15 to 18), and Treatment A (Period 3 from Day 18 - 32)
Sequence CABEXPERIMENTALSubjects receive three treatments (A: omaveloxolone 450 mg, B: omaveloxolone matching placebo and C: moxifloxacin 400 mg) administered in a single dose with the standard FDA high-fat meal in three periods. Treatment C (Period 1 from Day 1 - 4), Treatment A (Period 2 from Day 4 - 18), and Treatment B (Period 3 from Day 18 - 32)
Sequence CBAEXPERIMENTALSubjects receive three treatments (A: omaveloxolone 450 mg, B: omaveloxolone matching placebo and C: moxifloxacin 400 mg) administered in a single dose with the standard FDA high-fat meal in three periods. Treatment C (Period 1 from Day 1 - 4), Treatment B (Period 2 from Day 4 - 18), and Treatment A (Period 3 from Day 18 - 32)
Omaveloxolone only (Period 1), Then Omaveloxolone and efavirenz (Period 2)EXPERIMENTALPeriod 1 (Day 1 - 15): Omaveloxolone Capsules, 150 mg, administered orally in a single dose on Day 1 Period 2 (Day 15 - 43): Efavirenz Tablet, 600 mg, administered orally once daily from Day 15 - Day 42 and Omaveloxolone Capsules, 150 mg, administered orally in a single dose on Day 29
Group 1: matched healthy subjectsEXPERIMENTALOn the morning of Day 1, following an overnight fast of at least 10 hours, a single oral dose of 150 mg omaveloxolone (3 × 50 mg capsules) will be administered with 240 mL of water. No food will be allowed for 4 hours post dose. Pharmacokinetic samples will be obtained from pre dose until 336 hours post dose.
Group 2: subjects with mild hepatic impairmentEXPERIMENTALOn the morning of Day 1, following an overnight fast of at least 10 hours, a single oral dose of 150 mg omaveloxolone (3 × 50 mg capsules) will be administered with 240 mL of water. No food will be allowed for 4 hours post dose. Pharmacokinetic samples will be obtained from pre dose until 336 hours post dose.
Group 3: subjects with moderate hepatic impairmentEXPERIMENTALOn the morning of Day 1, following an overnight fast of at least 10 hours, a single oral dose of 150 mg omaveloxolone (3 × 50 mg capsules) will be administered with 240 mL of water. No food will be allowed for 4 hours post dose. Pharmacokinetic samples will be obtained from pre dose until 336 hours post dose.
Group 4: subjects with severe hepatic impairmentEXPERIMENTALOn the morning of Day 1, following an overnight fast of at least 10 hours, a single oral dose of 150 mg omaveloxolone (3 × 50 mg capsules) will be administered with 240 mL of water. No food will be allowed for 4 hours post dose. Pharmacokinetic samples will be obtained from pre dose until 336 hours post dose.
Omaveloxolone and Multiple Drugs (Part 1)EXPERIMENTALSingle oral doses of 2 mg midazolam, 1 mg repaglinide, 500 mg metformin, and a 10 mg rosuvastatin/0.25 mg digoxin cocktail on Days 1, 2, 3, and 5, respectively, and 18, 19, 20, and 22, respectively. Oral doses of 150 mg omaveloxolone on Days 12 to 27
Omaveloxolone & Gemfibrozil (Part 2)EXPERIMENTALSingle oral doses of 150 mg omaveloxolone on Days 1 and 13. Oral doses of 600 mg gemfibrozil (twice daily) on Days 10 to 18
Omaveloxolone and Itraconazole (Part 3)EXPERIMENTALSingle oral doses of 150 mg omaveloxolone on Days 1 and 13. Oral doses of 200 mg itraconazole on Days 10 to 18.
Omaveloxolone and Verapamil (Part 4)EXPERIMENTALSingle oral doses of 150 mg omaveloxolone on Days 1 and 13. Oral doses of 120 mg verapamil on Days 10 to 18.
Healthy Male SubjectsEXPERIMENTALSingle oral dose of 150 mg of \[14C\] omaveloxolone containing approximately 90 μCi as a capsule after an overnight fast of at least 10 hours.
Food Effect (Fasted)EXPERIMENTALSubjects will be randomly assigned to one of the two treatment sequences. Two single doses of omaveloxolone 150 mg (taken in multiple 50 mg capsules) will be administered to the subjects beginning in the fasted state (Period 1) with a crossover and then in the fed state (Period 2). Subjects will be confined beginning on Study Day -1 through the last PK blood draw on Study Day 6 during Period 1, and from Study Day 14 through the last PK blood draw on Study Day 20 during Period 2.
Effect (Fed)EXPERIMENTALSubjects will be randomly assigned to one of the two treatment sequences. Two single doses of omaveloxolone 150 mg (taken in multiple 50 mg capsules) will be administered to the subjects beginning in the fed state (Period 1) with a crossover and then in the fasted state (Period 2). Subjects will be confined beginning on Study Day -1 through the last PK blood draw on Study Day 6 during Period 1, and from Study Day 14 through the last PK blood draw on Study Day 20 during Period 2.
Dose ProportionalityEXPERIMENTALSubjects will be randomly assigned to one of two omaveloxolone dosages. A single dose of omaveloxolone (in either 50 mg or 100 mg) will be administered to the subjects in 50 mg capsules in a fasted state. Subjects will be confined beginning on Study Day -1 through the last blood sample collection on Study Day 6.
Omaveloxolone 5 mg & ipilimumabEXPERIMENTALOmaveloxolone (RTA 408) capsules, Dose1 taken orally once daily for 168 weeks, plus ipilimumab (3 mg/kg) administered at weeks 1, 4, 7, and 10.
Omaveloxolone 10 mg & ipilimumabEXPERIMENTALOmaveloxolone (RTA 408) capsules, 10 mg taken orally once daily for 168 weeks, plus ipilimumab (3 mg/kg) administered at weeks 1, 4, 7, and 10.
Omaveloxolone 5 mg & nivolumabEXPERIMENTALOmaveloxolone (RTA 408) capsules, 5 mg taken orally once daily for 168 weeks, plus nivolumab (240 mg) administered every two weeks as clinically indicated.
Omaveloxolone 10 mg & nivolumabEXPERIMENTALOmaveloxolone (RTA 408) capsules, 10 mg taken orally once daily for 168 weeks, plus nivolumab (240 mg) administered every two weeks as clinically indicated.
Omaveloxolone 20 mg & nivolumabEXPERIMENTALOmaveloxolone (RTA 408) capsules, 20 mg taken orally once daily for 168 weeks, plus nivolumab (240 mg) administered every two weeks as clinically indicated.
Omaveloxolone 100 mg & nivolumabEXPERIMENTALOmaveloxolone (RTA 408) capsules, 100 mg taken orally once daily for 168 weeks, plus nivolumab (240 mg) administered every two weeks as clinically indicated.
Omaveloxolone 150 mg & nivolumabEXPERIMENTALOmaveloxolone (RTA 408) capsules, 150 mg taken orally once daily for 168 weeks, plus nivolumab (240 mg) administered every two weeks as clinically indicated.
RTA 408 CapsulesEXPERIMENTALRTA 408 capsules, beginning dose 2.5 mg once daily, 28-day cycle, up to 12 cycles. Doses will increase by 100% of previous dose (e.g., 5 mg, 10 mg, 20 mg, etc.) until such time the Protocol Safety Review Committee decreases the escalation rate to 50% of the previous dose (e.g., 20 mg, 30 mg, 45 mg, etc). Dose escalation will continue until Maximum Tolerated Dose is identified.

Interventions

NameTypeDescription
OmaveloxoloneDRUGAdministered as specified in the treatment arm.
PlaceboDRUGAdministered as specified in the treatment arm.
Omaveloxolone capsules, 2.5 mgDRUG -
omaveloxolone capsules, 5 mgDRUG -
omaveloxolone capsules, 10 mgDRUG -
Placebo capsulesDRUG -
omaveloxolone capsules, 20 mgDRUG -
omaveloxolone capsules, 40 mgDRUG -
omaveloxolone capsules, 80 mgDRUG -
omaveloxolone capsules, 160 mgDRUG -
Omaveloxolone Capsules, 300 mgDRUG -
Omaveloxolone Capsules, 150 mgDRUG -
OmeprazoleDRUGOral tablet
MoxifloxacinDRUGMoxifloxacin capsules
EfavirenzDRUGEfavirenz Tablet, 600 mg, administered orally once daily
Omaveloxolone 50 mg capsulesDRUGCapsules containing 50 mg of omaveloxolone
Midazolam oral solutionDRUG2 mg/mL oral solution
Repaglinide 1 MGDRUG1 mg tablet
MetFORMIN 500 Mg Oral TabletDRUG500 mg tablet
RosuvastatinDRUG10 mg tablet
Digoxin tabletDRUG0.25 mg tablet
Gemfibrozil TabletsDRUG600 mg tablet
Itraconazole capsuleDRUG100 mg capsule
Verapamil PillDRUG120 mg tablet
[14C]-OmaveloxoloneDRUG\[14C\]-Omaveloxolone 50 mg capsules
Omaveloxolone Capsules (2.5 mg/capsule)DRUGCapsules containing 2.5 mg of omaveloxolone per capsule to the dosage indicated in the Arm Label
Ipilimumab (3 mg/kg)DRUGSterile solution containing ipilimumab to be delivered intravenously at 3mg/kg
Nivolumab (240 mg)DRUGSterile solution containing nivolumab to be delivered intravenously at 240 mg
Omaveloxolone Capsules (10 mg/capsule)DRUGCapsules containing 10 mg of omaveloxolone per capsule to the dosage indicated in the Arm Label
Omaveloxolone Capsules (50 mg/capsule)DRUGCapsules containing 50 mg of omaveloxolone per capsule to the dosage indicated in the Arm Label
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Eligibility Criteria

Age Range2 Years to 15 Years
SexALL
Healthy VolunteersNo
Study Sites34

Part 1: Key inclusion criteria: * Diagnosed with genetically confirmed Friedreich's Ataxia (FA), i.e., homozygous for guanine-adenine-adenine (GAA) repeat expansion in intron-1 of the frataxin gene, or GAA repeat expansion in 1 allele and with point mutations or deletions, or other non-GAA expansio...

Countries:United StatesAustraliaAustriaBrazilCanadaDenmarkFranceGermanyIndiaIrelandItalyNetherlandsSaudi ArabiaSpainTurkey (Türkiye)United Kingdom
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Recent Changes (Last 90 Days)

MEDIUMSep 3, 2026NCT06054893primaryCompletionDate: changed
MEDIUMSep 3, 2026NCT06054893primaryCompletionDate: changed
LOWJun 16, 2026NCT06953583lastUpdatePostDate: changed
LOWJun 16, 2026NCT06953583lastUpdatePostDate: changed
LOWJun 16, 2026NCT06953583lastUpdatePostDate: changed
MEDIUMJun 14, 2026NCT07297199TRIAL_REMOVED: changed
MEDIUMJun 14, 2026NCT07297199TRIAL_REMOVED: changed
MEDIUMJun 14, 2026NCT07297199TRIAL_REMOVED: changed

Frequently asked questions about Omaveloxolone

What is omaveloxolone?

Omaveloxolone is a small molecule being developed by Biogen Inc. (BIIB) for rare disease indications. It is currently in Phase 3 clinical development. The drug has been studied in multiple settings, including ocular inflammation and pain following surgery, melanoma, and healthy volunteer pharmacokinetic studies.

What is omaveloxolone used for?

Omaveloxolone is being investigated for use in several conditions. Clinical trials have evaluated it for inflammation and pain following ocular surgery, in patients with unresectable or metastatic melanoma, and in healthy subjects for pharmacokinetic and cardiac safety studies. Its primary therapeutic area is rare disease.

Who makes omaveloxolone?

Omaveloxolone is developed by Biogen Inc., a biotechnology company traded on NASDAQ under the ticker BIIB. Biogen is advancing the drug through clinical trials, with the most advanced studies in Phase 3.

What phase is omaveloxolone in?

Omaveloxolone is in Phase 3 clinical development. It has completed earlier phase trials, including Phase 1 and Phase 2 studies. The drug remains investigational and is not yet approved for any indication.

What clinical trials is omaveloxolone in?

Omaveloxolone has been studied in several completed clinical trials. NCT02065375 was a Phase 2 study in ocular inflammation and pain following surgery. NCT02259231 was a Phase 1 trial in melanoma. NCT03664453 and NCT05927649 were Phase 1 studies in healthy volunteers.

Is omaveloxolone the same as RTA 408?

Yes, omaveloxolone is also known as RTA 408. Clinical trial records refer to the drug as RTA 408 ophthalmic suspension in the ocular surgery study and as RTA 408 capsules in the melanoma trial. Both names refer to the same investigational compound.