Vonafexor ALPort Syndrome Efficacy & Safety TRIAl-1 (ALPESTRIA-1)

Inclusion Criteria:

• Signed informed consent (also for legal representatives, as applicable in the US forunder eighteen patients).

• Has confirmed diagnosis of Alport syndrome: clinical diagnosis (haematuria, familyhistory, hearing loss, ocular change) OR a kidney biopsy showing glomerular basementmembrane abnormalities consistent with AS, AND Genetic confirmation of AS.

• Has eGFR between ≥ 30 and < 90 ml/min/1.73m2.

• Has increased albuminuria criteria i.e. UACR ≥ 300 mg/g.

• If on an angiotensin converting enzyme inhibitor (ACEi) and/or angiotensin receptorblocker (ARB), should be on a stable well tolerated treatment during at least the 60days prior D1.

• If on Sodium-Glucose Transport Protein 2 (SGLT2), should be on stable well toleratedtreatment with SGLT2 during at least 60 days prior D1.

• If patient has a history of arterial hypertension, should be on stableanti-hypertensive therapy for at least 60 days prior to D1 and deemed controlled bythe investigator at screening and D1.

• Sexually active female subjects of childbearing potential and sexually mature malesubjects must use two acceptable effective methods of contraception for the entireduration of the study and for at least 6 weeks after last dose.

• Has negative results for hepatitis B surface antigen (HBsAg), hepatitis C virus (HCV) antibody, and human immunodeficiency virus (HIV).

• Is able to understand all study procedures in the informed consent form (ICF) andwilling to comply with all aspects of the protocol.

Exclusion Criteria:

• Is an employee of a site, clinical research organization, vendor, or sponsorinvolved with this study.

• Is pregnant or breastfeeding.

• Has participated in any investigational drug study within 60 days prior to D1.

• Any clinically significant illness within 30 days before D1 or surgical or medicalcondition (other than Alport syndrome) that could interfere with the subject's studycompliance; confound the study results; impact subject safety.

• Any history of active malignancy within the last 1 year before D1.

• Any other condition or circumstance that, in the opinion of the investigator, maymake the subject unlikely to complete the study or comply with study procedures andrequirements, or may pose a risk to the subject's safety and well-being.

• Has a history of an allergic condition that required the prescription of anemergency epinephrine injection (such as the EpiPen® Auto-Injector).

• Any prohibited co-medications within 30 days prior D1.

• Has ALT or AST above near normal (>1.5×ULN) at baseline.

• Are at high risk for atherosclerotic cardiovascular disease (ASCVD) risk, with anLDL-C level > 160 mg/dL (4.15 mmol/L) and subjects at intermediate risk for ASCVDrisk, with a LDL-C level > 190 mg/dL (4.91 mmol/L).

• Has moderate or severe hepatic impairment (Child-Pugh score B or C).

• Is taking CYP3A4/5 inhibitors or inducers.