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EXP001990

Paper

Bi-specific splice-switching PMO oligonucleotides conjugated via a single peptide active in a mouse model of Duchenne muscular dystrophy (2015)

Peptide

Bi-specific D3 (in vivo)

Sequence: RXRRBRRXRYQFLIRXRBRXRB

RNA

PMO (phosphorodiamidate morpholino oligomer; splice-switching oligonucleotide)

All experiment fields

Experiment Id EXP001990
Paper Bi-specific splice-switching PMO oligonucleotides conjugated via a single peptide active in a mouse
Peptide Bi-specific D3 (in vivo)
Delivery Success Class yes
In Vivo Flag yes
Uptake Confirmed no
Label Confidence high
In Vitro Functional Effect
Endosomal Escape Evidence
Peptide Concentration 0.5 nmol conjugate per TA.
Rna Concentration Contains 2 PMOs per conjugate (total PMO equivalents delivered in the conjugate).
Mixing Ratio Bi-specific conjugate (both PMOs on one Pip6a; click linkage for second PMO).
Formulation Format Covalent CPP–PMO conjugate(s) for in vivo splice switching in muscle (intramuscular administration).
Formulation Components D3 bi-specific Pip6a–PMO–PMO conjugate.
Size Nm
Zeta Mv
Model Scope in_vivo
Model Type in vivo
Cell Lines Or Primary Cells
Animal Model mdx mice (C57BL/10ScSn-Dmdmdx/J); tibialis anterior muscle intramuscular injection
Administration Route Intramuscular injection into TA: 0.5 nmol peptide–PMO in 30 µL 0.9% saline; analysis 2 weeks post-injection
Output Type In vivo functional RNA delivery: exon skipping in TA muscle (RT-PCR + qPCR)
Output Value ~35–40% Dmd exon23 skipped transcripts by qPCR; Acvr2b exon5 skipping present (lower than Dmd).
Output Units
Output Notes Comparable in vivo splice-switching activity to D2 and to cocktail treatment.
Toxicity Notes
Curation Notes