The study will evaluate safety and efficacy of intrathecal delivery of GC101 gene therapy drug as a treatment of spinal muscular atrophy Type 1 (SMA 1) patients.
The purpose of this trial is to evaluate safety and efficacy of gene therapy drug GC101 in SMA 1 patients. Open-label, dose-escalation clinical trial of GC101 will be conducted in multiple centers in China. GC101 will be administrated intrathecally. Short-term safety will be evaluated in 52 weeks and enter long-term follow-up study of 5 years at will. Patients will be tested at baseline and followed up on various time points. The primary analysis for efficacy will be assessed when all patients reach 18 months of age on the motor milestone of sit unassisted for at least 10 seconds.
Study Type
INTERVENTIONAL
Allocation
NON_RANDOMIZED
Purpose
TREATMENT
Masking
NONE
Enrollment
18
Self-complementary AAV9 carrying a codon-optimized SMN coding sequence(coSMN1) driven by CMV enhancer and chicken β-actin promoter
Peking University, First Hospital, Department of Pediatrics
Beijing, China
RECRUITINGBayi Children's Hospital, Seventh Medical Center, PLA general hospital
Beijing, China
RECRUITINGShengjing Hospital of China Medical University
Shenyang, China
Incidence of Treatment-Emergent Adverse Events [Safety and Tolerability]
Frequency of treatment-related adverse events (AEs), serious adverse events (SAEs), and changes from baseline in relevant clinical laboratory tests
Time frame: when patient reaches 18 months of age
Proportion of patients treated with GC101 who achieve motor milestone of sit unassisted for at least 10 seconds at 18 months of age
Time frame: when patient reaches 18 months of age
Ability to thrive
Ability to thrive is defined as the following at 18 months of age: does not receive nutrition through mechanical support or other non-oral method; maintains weight
Time frame: when patient reaches 18 months of age
Changes from baseline Children's hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP-INTEND) score
CHOP INTEND score ranges from 0 to 64 with higher scores indicating higher motor function
Time frame: when patient reaches 18 months of age
Proportion of event-free survival patients
Time frame: when patient reaches 14 months of age
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Children's Hospital of Soochow University
Suzhou, China
RECRUITING