Gene Transfer Therapy for Treating Children and Adults With Limb Girdle Muscular Dystrophy Type 2D (LGMD2D)

Inclusion Criteria: * Six LGMD2D subjects ages 5 and older based on the clinical degree of involvement (impaired muscle function/weakness, sufficient muscle preservation) * Preservation of EDB muscle or another muscle if judged more favorable because of adequate muscle mass for gene transfer * Males and females of any ethnic group * Established mutations of an -SG gene on both alleles * Ability to cooperate for testing * Sexually active patients must be willing to practice a reliable method of contraception during the study Exclusion Criteria: * Active viral infection (symptoms listed in section 9.0 of the protocol) * LGMD2D subjects without weakness or functional loss * Cardiomyopathy based on clinical exam and ECHO with ejection fraction less than 40% * HIV infected * Hepatitis A, B, or C infected * Autoimmune diseases and immunosuppressive drugs (other than pulse methylprednisolone at time of gene transfer) * Persistent leucopenia or leucocytosis (WBC less than or equal to 3.5 K/cu mm or at least 20.0 K/ cu mm) or neutrophils less than 1.5 K/ cu mm * Concomitant illness or requirement for chronic drug treatment that in the opinion of the Principal Investigator creates unnecessary risks for gene transfer * Pregnancy * Abnormal laboratory values considered clinically significant * Alcoholism (CAGE questionnaire), and laboratory tests such as GGT and MCV