IP Library Granted Patent US 11,534,501
Granted Patent B2
US 11,534,501 · App. 16/757,207 · Granted Dec 27, 2022

Adeno-associated virus vector delivery of muscle specific micro-dystrophin to treat muscular dystrophy

Inventors: Louise Rodino-Klapac (E. Groveport, OH); Jerry R. Mendell (Columbus, OH)
Assignee: RESEARCH INSTITUTE AT NATIONWIDE CHILDREN'S HOSPITAL
A61K48/0058A61P21/00C12N15/86
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Quick Facts
Patent No.
US 11,534,501
App. No.
16/757,207
Granted
Dec 27, 2022
Kind
B2
Abstract

The invention provides gene therapy vectors, such as adeno-associated vims (AAV) vectors, expressing a miniaturized human micro-dystrophin gene and method of using these vectors to express micro-dystrophin in skeletal muscle including diaphragm and cardiac muscle and to protect muscle fibers from injury, increase muscle strength and reduce and/or prevent fibrosis in subjects suffering from muscular dystrophy.

Claims (16)

1. A method of treating muscular dystrophy comprising administering i) a therapeutically effective amount of a recombinant AAV vector comprising nucleotide 236 to nucleotide 4842 of SEQ ID NO: 3 and ii) a therapeutically effective amount of a recombinant AAV vector comprising nucleotides 120 to 2091 of SEQ ID NO: 6.

2. A method of increasing muscular force or muscle mass in a subject suffering from muscular dystrophy comprising administering i) a therapeutically effective amount of a recombinant AAV comprising nucleotide 236 to nucleotide 4842 of SEQ ID NO: 3 and ii) a therapeutically effective amount of a recombinant AAV vector comprising nucleotides 120 to 2091 of SEQ ID NO: 6.

3. A method of reducing or preventing fibrosis in a subject suffering from muscular dystrophy comprising administering i) a therapeutically effective amount of i) a recombinant AAV vector expressing comprising nucleotide 236 to nucleotide 4842 of SEQ ID NO: 3 and ii) a therapeutically effective amount of a recombinant AAV vector comprising nucleotides 120 to 2091 of SEQ ID NO: 6.

4. The method of claim 1 wherein the muscular dystrophy is Duchenne muscular dystrophy.

5. The method of claim 1 wherein at least one of the recombinant AAV vectors is the serotype AAVrh.74, AAV1, AAV2, AAV4, AAV5, AAV6, AAV7, AAV8, AAV9, AAV10, AAV11, AAV12 or AAV13.

6. The method of claim 1 wherein at least one of the recombinant AAV vectors is administered by intramuscular injection or intravenous injection.

7. The method of claim 1 wherein at least one of the recombinant AAV vectors is administered systemically.

8. The method of claim 7 , wherein at least one of the recombinant AAV vectors is parenterally administered by injection, infusion or implantation.

9. The method of claim 2 wherein at least one of the recombinant AAV vectors is the serotype AAVrh.74, AAV1, AAV2, AAV4, AAV5, AAV6, AAV7, AAV8, AAV9, AAV10, AAV11, AAV12 or AAV13.

10. The method of claim 2 wherein at least one of the recombinant AAV vectors is administered by intramuscular injection or intravenous injection.

11. The method of claim 2 wherein at least one of the recombinant AAV vectors is administered systemically.

12. The method of claim 11 , wherein at least one of the recombinant AAV vectors is parenterally administered by injection, infusion or implantation.

13. The method of claim 3 wherein at least one of the recombinant AAV vectors is the serotype AAVrh.74, AAV1, AAV2, AAV4, AAV5, AAV6, AAV7, AAV8, AAV9, AAV10, AAV11, AAV12 or AAV13.

14. The method of claim 3 wherein at least one of the recombinant AAV vectors is administered by intramuscular injection or intravenous injection.

15. The method of claim 3 wherein at least one of the recombinant AAV vectors is administered systemically.

16. The method of claim 15 , wherein at least one of the recombinant AAV vectors is parenterally administered by injection, infusion or implantation.

Assignments (1)
ASSIGNMENT OF ASSIGNOR'S INTEREST Recorded Oct 2, 2020
From: RODINO-KLAPAC, LOUISE; MENDELL, JERRY R.
To: RESEARCH INSTITUTE AT NATIONWIDE CHILDREN'S HOSPITAL
Reel/Frame 053962/0065 →
Continuity (2)
Provisional Application 62573955 · Oct 18, 2017
Related Publication 20210128749A1 · May 6, 2021