GV101 for Friedreich's Ataxia (FA) Treatment

Inclusion Criteria: 1. Be male or female aged 16 - 40 years, inclusive, at the time of informed consent. 2. Have a genetically confirmed diagnosis of FA with homozygous GAA repeat expansions. 3. Have mFARS score ≥20 and ≤80. The screening and baseline mFARS values must fall within this range; these two values also must be within 4.5 points of each other. 4. Have an hs-c-troponin I above the laboratory defined 99th percentile at screening and at least 14 ng/L. 5. Be able to ambulate with or without assistance (If a participant uses an assistive device for the walk test on V1, the same or similar assistive device must be used on all subsequent walk tests throughout the trial). 6. Be able to sit upright. 7. Be willing and able to comply with all study procedures. 8. Heterosexually active women of child-bearing potential and males who are sexually active with female(s) of child-bearing potential must agree to highly effective contraception from screening until at least 30 days after the last dose of IMP for women and 90 days after the last dose of IMP for men. Acceptable highly effective methods include: * Contraceptive implant. * Intrauterine device (IUD), including hormonal or copper IUD. * Bilateral salpingectomy or bilateral tubal occlusion (including tubal ligation). * Vasectomized male partner(s) (6 months from vasectomy) with documented azoospermia. 9. Provide written informed consent. Exclusion Criteria: 1. Be pregnant or lactating. 2. Have cognitive impairment that may interfere with compliance with study procedures. 3. Have swallowing impairment(s) that preclude IMP dosing. 4. Have significant cardiac disease, apart from mild/moderate cardiomyopathy associated with FA, including but not limited to severe or unstable heart failure (NYHA Class III/IV), unstable angina or recent myocardial infarction, significant arrhythmias, left-sided structural heart disease, valvular disease, pericardial constriction, or restrictive or congestive cardiomyopathy. 5. Have a history of significant QTcF prolongation or QTcF \> 470 ms (females) or \> 450 ms (males) at screening. 6. Have other active neurological diseases. 7. Have known untreated thyroid disease, or an abnormal thyroid panel at screening that is concerning for disease. 8. Have moderate or severe hepatic or renal impairment, including but not limited to eGFR \< 60 mL/min/1.73 m² (by 2021 CKD-EPI) or creatinine clearance (CLcr- calculated by Cockcroft-Gault) \>1.5 ULN or AST/ALT or total bilirubin \>1.5x ULN at screening; retest allowed if abnormalities are potentially attributable to muscle injury. 9. Have uncontrolled diabetes, defined as HbA1c \> 7.0%. 10. Have albumin below LLN (screening). 11. Have active infections or test positive for HIV, hepatitis B, or hepatitis C at screening. 12. Have clinically significant hematology or biochemistry abnormalities or any laboratory result or medical condition that, in the investigator's judgment, would increase patient risk. 13. Have a history of known or suspected drug or alcohol abuse within the last 2 years. 14. Participants currently receiving omaveloxolone are not eligible. Participants who have previously received omaveloxolone must complete a minimum of 4 week washout period prior to screening. Other background medications or supplements intended to treat FA must be on stable doses for ≥30 days before screening and be intended to remain unchanged throughout the 12-week trial treatment period. 15. Be taking medications with narrow therapeutic indices that are primarily metabolized by CYP2C8, CYP2C9, or CYP2C19 and cannot be reduced in dose; be taking medications with narrow therapeutic indices that are p-gp substrates. Be taking daily standard doses of strong inducers or inhibitors of CYP3A4 that cannot be modified. 16. Be taking dimethylfumerate or other medicinal products or supplements intended to raise FXN currently, within the past 45 days or within 5 half-lives of the medicinal product, whichever is longer 17. Have participated in any other interventional clinical trial or received any investigational drug in any other clinical trial within 30 days or 5 half-lives of the IMP, whichever is longer, prior to the Baseline visit. 18. Have undergone prior gene therapy for FA. 19. Have previously documented mitochondrial respiratory chain disease. 20. Have scheduled surgery for scoliosis or pes cavus during the clinical trial period. 21. Current or prior active suicidal ideation with intent or plan or suicidal behavior/attempt (within 6 months) at screening or between screening and baseline.