A Study to Evaluate the Safety of AB-1003 (Previously LION-101) in Subjects With Genetic Confirmation of LGMD2I/R9 (Part1)

Inclusion Criteria:

1. Male and female subjects aged 18 and 65 years with clinical diagnosis of LGMD2I/R9and confirmation of FKRP gene mutation.

2. Ability to walk/run 10 meters in <30 seconds.

3. Able to understand and comply with all study procedures.

4. Sexually active females of childbearing potential and female and male partners ofmale subjects receiving study intervention must use a barrier method ofcontraception for the first 6 months after dosing.

Exclusion Criteria:

1. Significant cardiomyopathy as defined by echocardiogram (left ventricular ejectionfraction <40%), evidence of conduction defect (increased PR and RR intervals, leftbundle branch block and QTcF >480m/sec), NYHA Class 3 or 4 heart failure, or MRIgadolinium enhancement evidence of clinically important myocardial fibrosis.

2. Contraindication to MRI or hypersensitivity to contrast dyes, shellfish or iodine.

3. Implanted spinal rods, cardiac pacemaker or other implantation that would distortcardiac MRI images.

4. History of active, ongoing chronic liver disease (e.g. hepatitis, HIV-related liverdisease, hemochromatosis, steatosis, etc.) or abnormal liver function tests (abnormal GGT and/or abnormal total/direct bilirubin >upper limit of normal [ULN]and/or elevated AST and ALT >2 ULN).

5. Abnormal renal function (GFR <60 ml/min, using the Modification of Diet in RenalDisease equation).

6. Any life-threatening disease, including malignant neoplasms and medical history ormalignant neoplasms within the past 5 years prior to screening (except basal andsquamous cell skin cancer).

7. In the opinion of the investigator, a pre-existing medical condition thatpredisposes the subject to risks that outweighs the potential benefits.

8. Requirement for daytime ventilatory support.

9. Change in glucocorticosteroid treatment within 3 months prior to screening visit.

10. Exposure to another investigational drug within 3 months prior to study treatment orany previous treatment with gene therapy.

11. Ongoing participation in any other therapeutic clinical trial.

12. Neutralizing antibody titer to AAV9 >1:5.

13. Female subjects who are pregnant, plan to become pregnant in the next 12 months, orbreastfeeding.