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Exploratory Study of NS-065/NCNP-01 in Duchenne muscular dystrophy

Phase 1
Conditions
Duchenne muscular dystrophy
Registration Number
JPRN-UMIN000010964
Lead Sponsor
ational Center of Neurology and Psychiatry
Brief Summary

Komaki.H, Nagata.T, Saito.T, Masuda.S, Takeshita.E, Sasaki.M, Tachimori.H, Nakamura.H, Aoki.Y, Takeda.S Systemic administration of the antisense oligonucleotide NS-065/NCNP-01 for skipping of exon 53 in patients with Duchenne muscular dystrophy. Sci Transl Med. 2018, 10(437) https://stm.sciencemag.org/content/10/437/eaan0713.short

Detailed Description

Not available

Recruitment & Eligibility

Status
Complete: follow-up complete
Sex
Male
Target Recruitment
10
Inclusion Criteria

Not provided

Exclusion Criteria

Subject with Duchenne muscular dystrophy meeting any of the following criteria must not be enrolled in the study: 1)Has participated in other pharmacological clinical trial that might recover dystrophin protein by the readthrough or the exon-skipping therapy, and/or upregulate the dystrophin-associated proteins such as utrophin. 2) A forced vital capacity (FVC) < 50% of predicted. 3) A left ventricular ejection fraction (EF) < 40% or fractional shortening (FS) < 25% based on echocardiogram (ECHO). 4) Surgery within the last 3 months prior to the first anticipated administration of study medication or planned for anytime during the duration of the study. 5) Positive hepatitis B surface antigen (HbsAg), hepatitis C antibody test (HCV), or human immunodeficiency virus (HIV) test at screening. 6) Current diagnosis of any immune deficiency or autoimmune disease. 7) Current diagnosis of any active or uncontrolled infection, cardiomyopathy, or liver or renal disease. 8) Use of any other investigational agents and/or experimental agents within 3 months prior to the first anticipated administration of study medication. 9) History of any severe drug allergy. 10) Unable to give informed consent about using adequate contraception from the first administration until at least 6 months after the last dose of study medication, by parent(s) or legal guardian. 11) Subject considered by the investigator (or sub-investigator), for any reason, to be an unsuitable candidate for the study.

Study & Design

Study Type
Interventional
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
Safety (adverse event and adverse drug reaction)
Secondary Outcome Measures
NameTimeMethod
1. Expression of dystrophin protein 2. Detection of exon53 skipped mRNA of dystrophin 3. NS-065/NCNP-01 concentration of the blood plasma 4. NS-065/NCNP-01 concentration of the urine 5. Serum Creatine kinase concentration
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