• Participants and/or their parent(s)/guardian(s) must have given written informed consent before any study-related activities are carried out and must be able to understand the full nature and purpose of the trial, including possible risks and adverse effects. Assent, where appropriate, will be obtained according to institutional guidelines.

• Males and females, 8 years of age and above.

• Have a clinical diagnosis of OPA1 mutation (haploinsufficiency) associated ADOA.

• No other ocular pathology.

• Patients with best-corrected visual acuity (BCVA) of between 20/40 (70 Early Treatment of Diabetic Retinopathy Study \[ETDRS\] letters) and 20/160 (39-43 ETDRS letters)

• Willing and able to comply with all study assessments and adhere to the protocol schedule and restrictions.

• For sites performing the Detection of apoptosis in retinal cells (DARC) procedure, and in volunteers ≥ 12 years only:

‣ Female volunteers must:

‣ I. Be of non-child-bearing potential at least 6 weeks before the screening visit or postmenopausal (where postmenopausal is defined as no menses for 12 months without an alternative medical cause), or

‣ II. If of childbearing potential, must:

∙ Have a negative pregnancy test at the screening visit and prior to each administration of ANX776, and

∙ Agree not to attempt to become pregnant or donate ova from signing the consent form until at least 30 days after the last dose of ANX776, and

∙ Agree to use adequate contraception (defined as the use of a condom by the male partner combined with the use of a highly effective method of contraception from one month prior to screening until at least 30 days after the last dose of ANX776, if not exclusively in a same-sex relationship or abstinent as a committed lifestyle.

⁃ Male volunteers must:

• Agree not to donate sperm from signing the consent form until at least 90 days after the last dose of ANX776, and

∙ If engaging in sexual intercourse with a female partner who could become pregnant, agree to use adequate contraception (defined as the use of a condom combined with the use of a highly effective method of contraception) from signing the consent form until at least 90 days after the last dose of study drug.