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

This study is testing a new gene therapy called AB-1003 for adults with limb-girdle muscular dystrophy type 2I/R9 (LGMD2I/R9), a genetic muscle disease caused by mutations in the FKRP gene. The therapy aims to restore a missing protein to improve muscle function. You may be eligible if... - You are between 18 and 65 years old - You have been diagnosed with LGMD2I/R9 confirmed by genetic testing of the FKRP gene - You can walk or run 10 meters in less than 30 seconds - You are willing to use barrier contraception for 6 months after receiving treatment You may NOT be eligible if... - You have significant heart muscle disease (ejection fraction below 40%, serious electrical abnormalities, or NYHA class 3 or 4 heart failure) - You have an MRI contraindication or allergy to contrast agents, shellfish, or iodine - You have a cardiac pacemaker or implanted spinal rods - You have active liver disease or abnormal liver function tests - You have reduced kidney function (GFR below 60) - You have had cancer within the past 5 years (excluding minor skin cancers) - You require daytime ventilator support - You have high neutralizing antibodies to the viral vector used (AAV9 titer above 1:5) - You have previously had gene therapy - You are pregnant or breastfeeding Talk to your doctor to see if this trial is right for you.