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Phase 2 N=19 Randomized Quadruple-blind Treatment

A First in Human Study of RT001 in Patients With Friedreich's Ataxia

Friedreich's Ataxia

Enrolled (actual)
19
Serious AEs
5.3%
Results posted
Nov 2020
Primary outcome: Primary: Number of Patients With Adverse Events — 5; 2; 5; 2 participants

Study Design & Population

Study type
Interventional
Phase
Phase 2
Interventions
Low dose cohort (Drug); High dose cohort (Drug)
Age
Adult · 18+ yrs
Sex
All
Sponsor
Biojiva LLC
Primary completion
Jun 2016

Outcome Measures

OutcomeResultp-value
PRIMARY
Number of Patients With Adverse Events
5; 2; 5; 2
SECONDARY
Pharmacokinetics - Area Under the Concentration-time Curve After a Single Dose
524; 915
SECONDARY
Pharmacokinetics - Maximum Observed Plasma Concentration After a Single Dose
35.9; 58.6
SECONDARY
Pharmacokinetics - Time to Reach Maximum Plasma Concentration After a Single Dose
6.05; 8.0
SECONDARY
Pharmacokinetics - Maximum Observed Plasma Concentration After Final Dose on Day 28
91; 359.9
SECONDARY
Pharmacokinetics - Terminal Half-life Estimation After Final Dose on Day 28
42.3; 30.3
SECONDARY
Change From Baseline at 28 Days in the Timed 25 Foot Walk (T25FW)
.503; -1.87; -7.475; .608
SECONDARY
Change From Baseline at 28 Days in the Friedreich Ataxia Rating Scale (FARS) - Neurological Score (Minimum Score 0, Maximum Score 125, Lower is Better)
-6.22; -6.47; -3.3; -2
SECONDARY
Change From Baseline at 28 Days in Peak Workload for the Treated Population vs. the Comparator Population
.08; -.08 .008 sig

Summary

The purpose of this study is to evaluate the safety, tolerability and pharmacokinetics of RT001 in patients with Friedreich's ataxia.

Eligibility Criteria

Inclusion Criteria

  • Male or female 18 to 50 years of age
  • Medical history consistent with the symptoms of FRDA at ≤ 25 years of age
  • Homozygous for GAA repeat expansions in the Frataxin gene in the affected range for FRDA
  • FARS-Neurological score of 20-90 points
  • Ambulatory (with or without assistive device) and capable of performing assessments/evaluations
  • Body Mass Index ≤ 29.9 kg/m2
  • Agrees to dietary restrictions and agrees to receive calls from a dietary coach
  • Signed the informed consent form prior to entry into the study
  • Agrees to spend the required number of overnight clinic days
  • Able to provide the necessary repeated blood samples

Exclusion Criteria

  • Received treatment with other experimental therapies within the last 30 days prior to the first dose
  • Known point mutation in the FXN gene
  • History of malignancies (other than basal cell carcinomas)
  • Impaired renal function at screening
  • Alanine transaminase (ALT) or aspartate transaminase (AST) laboratory values > 2 x upper limit of normal (ULN) at screening
  • Known hepatitis B surface antigen (HBsAg)-positive, or known or suspected active hepatitis C infection, or is known to be human immunodeficiency virus (HIV) positive
  • Female who is breastfeeding or has a positive pregnancy test
  • Male participant or female participant of child bearing potential, who is sexually active and unwilling/unable to use a medically acceptable and effective double barrier birth control method throughout the study
  • Unwilling or unable to comply with the requirements of the protocol
  • Clinically significant cardiac abnormalities at screening that, in the opinion of the Investigator, would make the patient unsuitable for enrollment
  • Diabetes mellitus (Type 1 or 2)
  • Suicidal ideation as determined by the Columbia-Suicide Severity Rating Scale
  • History, within the last 2 years, of alcohol abuse, significant mental illness, or physical opioid dependence
  • Cannot adhere to the dietary guidance required to be followed by the protocol
  • Cannot take the medication due to impairment in swallowing capsules
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT02445794). 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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