Patient description: The patient is a 14-year-old boy complaining of scoliosis and back pain. He has no other medical condition. He used to be able to play routine activities such as basketball and soccer, however, he recently has problem doing them. The pain is in his leg and back and aggravated by physical activities. He prefer lying down most of the time. He is not happy with his body gesture and complaints of shoulder imbalance and shifting his head to right. Patient is a well-dressed and well-nourished adolescent who is alert and cooperative. The left shoulder is slightly higher than the right shoulder. The scoliotic curve is measured as 45 degree. The patients is candidate for scoliosis surgery according to perioperative MEP monitoring

Clinical trial eligibility criteria: eligible ages (years): 7.0-999.0,  Duchenne Muscular Dystrophy Be a male with DMD with a mutation that may be amenable to exon 53 skipping as documented by a genetic report from an accredited laboratory confirming mutation endpoints by multiplex ligation-dependent probe amplification. 2. Be 7 years of age or older. 3. Has been on a stable dose of oral corticosteroids for at least 24 weeks prior to study drug administration and the dose is expected to remain constant (except for modifications to accommodate changes in weight) throughout the study or has not received corticosteroids for at least 24 weeks prior to study drug administration and does not expect to start corticosteroids throughout the study. 4. Be unable to ambulate ("non-ambulatory"). By definition, loss of ambulation means patient or caregiver reported continuous wheelchair use that has been verified by a clinical evaluator. The following conditions should be met: 1. Condition is not secondary to acute worsening of mobility due to orthopedic morbidity (eg, fracture, sprain, or injury) or surgical procedure. 2. Unable to perform 10-meter walk run test. 5. Has stable pulmonary function that, in the opinion of the Investigator, is unlikely to decompensate over the study period. 6. Patients who are post-pubertal and sexually active must agree to use, for the entire duration of the study and for 90 days post last dose, a male condom and the female sexual partner must also use a medically acceptable form of birth control (eg, oral contraceptives). 7. Able to understand and comply with all study requirements, in the Investigator's opinion, or if under the age of 18 years, must have a parent(s) or legal guardian(s) who is able to understand. 8. Willing to provide informed consent to participate in the study, or if under the age of 18 years, be willing to provide informed assent, if applicable, and have a parent(s) or legal guardian(s) who is willing to provide informed consent for the patient to participate in the study  Use of any pharmacologic treatment (other than corticosteroids) within 12 weeks of study drug administration that in the opinion of the Investigator might have an effect on skeletal muscle strength or function (eg, growth hormone, anabolic steroids). 2. Previous treatment with any investigational drug or exon skipping therapy within the last 3 months. 3. Major change in physiotherapy regimen within the past 3 months or expected change over the study period. 4. Major surgery within 3 months of study drug administration or planned major surgery for any time during this study. 5. Presence of other clinically significant illness that cannot be attributed to classic Duchenne disease course including significant cardiac, pulmonary, hepatic, renal, hematologic, immunologic, behavioral disease, or malignancy. 6. Systemic use of any aminoglycoside antibiotic within 12 weeks of study drug administration or anticipated need for use of an aminoglycoside antibiotic or statin during the study. 7. Must not require antiarrhythmic and/or antidiuretic therapy for heart failure. Patients are allowed to take other medication including angiotensin-converting enzyme (ACE) inhibitors, angiotensin receptor blocking agents, β blockers or potassium, provided they have been on a stable dose for 24 weeks prior to study drug administration and the dose is expected to remain constant throughout the study. 8. If the patient is asymptomatic but has a LVEF < 40% at Screening or clinically significant at the discretion of Investigator, the Investigator should discuss of patient in the study with the appropriate institutional safety committee or medical monitor. 9. Prior or ongoing medical condition that could, in the Investigator's opinion, adversely affect the safety of the patient, make it unlikely that the course of treatment would be completed, or impair the assessment of study results