US2023183696A1PendingUtilityA1

Compositions and Methods for Modulation of SMN2 Splicing in a Subject

Assignee: BIOGEN MA INCPriority: Apr 17, 2014Filed: Nov 22, 2022Published: Jun 15, 2023
Est. expiryApr 17, 2034(~7.7 yrs left)· nominal 20-yr term from priority
C12N 2310/343C12N 2310/346C12N 2320/33C12N 2310/11C12N 2310/3341C12N 2310/315C12N 15/113C12N 2310/322
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Claims

Abstract

Disclosed herein are compounds, compositions and methods for modulating splicing of SMN2 mRNA in a subject.

Claims

exact text as granted — not AI-modified
1 . A compound comprising a modified oligonucleotide consisting of 16-20 linked nucleosides, wherein the modified oligonucleotide is complementary to SMN2; and wherein each internucleoside linkage is either phosphorothioate or phosphodiester and at least one internucleoside linkage is phosphorothioate and at least one internucleoside linkage is phosphodiester. 
     
     
         2 . The compound of  claim 1 , wherein the modified oligonucleotide has a nucleobase sequence selected from any one of SEQ ID NOs: 1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 26, or 27, and having 6 or more phosphodiester internucleoside linkages, wherein each internucleoside linkage that is not a phosphodiester internucleoside linkage is a phosphorothioate internucleoside linkage. 
     
     
         3 . (canceled) 
     
     
         4 . (canceled) 
     
     
         5 . (canceled) 
     
     
         6 . The compound of  claim 1 , having 7, 8, 9, 10, or more phosphodiester internucleoside linkages, wherein each internucleoside linkage that is not a phosphodiester internucleoside linkage is a phosphorothioate internucleoside linkage. 
     
     
         7 . (canceled) 
     
     
         8 . (canceled) 
     
     
         9 . (canceled) 
     
     
         10 . (canceled) 
     
     
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         43 . (canceled) 
     
     
         44 . The compound of  claim 1 , wherein each nucleoside of the modified oligonucleotide comprises a modified sugar moiety, and wherein each modified sugar moiety comprises a 2′-O-methoxyethyl modification. 
     
     
         45 . A pharmaceutical composition comprising the compound of  claim 1 , and a pharmaceutically acceptable carrier. 
     
     
         46 . A method of promoting inclusion of exon 7 in SMN2 transcripts in a cell, tissue or organ, comprising contacting said cell, tissue or organ with the compound of  claim 1 . 
     
     
         47 . A method of treating Spinal Muscular Atrophy Type I, Spinal Muscular Atrophy Type II, Spinal Muscular Atrophy Type III, or Spinal Muscular Atrophy Type IV, comprising administering a therapeutically effective amount of the compound of  claim 1  to a patient in need thereof. 
     
     
         48 . (canceled) 
     
     
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         108 . (canceled) 
     
     
         109 . An antisense oligonucleotide consisting of the nucleic acid sequence set forth in SEQ ID NO: 1, 2, or 7, wherein all cytosine residues of the nucleic acid sequence are 5-methylcytosines, and wherein each nucleoside of the nucleic acid sequence comprises a 2′-O-methoxyethyl sugar modification. 
     
     
         110 . The antisense oligonucleotide of  claim 109 , wherein the nucleic acid sequence has any one of the following backbone chemistries:
 (i) sossssossssossssoss;   (ii) sosososososososooss;   (iii) soosossossossosooss;   (iv) sossossossossosooss;   (v) sossossossossossoss; or   (vi) sossosssosssosssoss,   wherein “s” indicates a phosphorothioate linkage and “o” indicates a phosphodiester linkage.   
     
     
         111 . A pharmaceutical composition comprising the antisense oligonucleotide of  claim 109  and a pharmaceutically acceptable carrier. 
     
     
         112 . A method of treating spinal muscular atrophy (SMA) in a human subject, the method comprising administering to the human subject a therapeutically effective amount of the antisense oligonucleotide of  claim 109 . 
     
     
         113 . The method of  claim 112 , wherein the SMA is SMA Type I, SMA Type II, SMA Type III, or SMA Type IV.

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