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EXP001212

Paper

Sustained Dystrophin Expression Induced by Peptide-conjugated Morpholino Oligomers in the Muscles of mdx Mice (2008)

Peptide

CPP-B conjugate (M23D-B, dystrophin-targeting PPMO)

Sequence: RXRRBRRXRRBRXB

RNA

PMO (morpholino antisense oligo)

All experiment fields

Experiment Id EXP001212
Paper Sustained Dystrophin Expression Induced by Peptide-conjugated Morpholino Oligomers in the Muscles of
Peptide CPP-B conjugate (M23D-B, dystrophin-targeting PPMO)
Delivery Success Class yes
In Vivo Flag yes
Uptake Confirmed no
Label Confidence high
In Vitro Functional Effect
Endosomal Escape Evidence
Peptide Concentration
Rna Concentration 12 mg/kg/day (PPMO dose) for 4 days
Mixing Ratio
Formulation Format covalent peptide–PMO conjugate (PPMO)
Formulation Components CPP-B conjugated to PMO M23D (exon 23 skipping)
Size Nm
Zeta Mv
Model Scope in_vivo
Model Type in vivo
Cell Lines Or Primary Cells
Animal Model mdx mouse (DMD model)
Administration Route intravenous (IV)
Output Type exon skipping / dystrophin restoration / pathology biomarkers
Output Value Sustained exon 23 skipping; dystrophin restoration in skeletal + cardiac muscle; serum CK markedly reduced.
Output Units
Output Notes Nested RT-PCR showed exon 23 skipping in heart/diaphragm/quadriceps up to 9 weeks; dystrophin detected by in-gel western and immunofluorescence; reduced inflammatory infiltration in heart (Figs. 3–5).
Toxicity Notes No detectable toxicity reported; clinical chemistry (BUN/creatinine/AST/ALT/CK) monitored.
Curation Notes