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EXP002296

Paper

Systemic peptide-mediated oligonucleotide therapy improves long-term survival in spinal muscular atrophy (2016)

Peptide

Pip6a

Sequence: Ac-(RXRRBRRXRYQFLIRXRBRXRB)-COOH

RNA

PMO (splice-switching antisense oligonucleotide)

All experiment fields

Experiment Id EXP002296
Paper Systemic peptide-mediated oligonucleotide therapy improves long-term survival in spinal muscular atr
Peptide Pip6a
Delivery Success Class yes
In Vivo Flag yes
Uptake Confirmed yes
Label Confidence very high
In Vitro Functional Effect
Endosomal Escape Evidence
Peptide Concentration 2.5–10 µg/g (PND0/PND2)
Rna Concentration 2.5–10 µg/g
Mixing Ratio 1:1 covalent conjugate
Formulation Format peptide–PMO conjugate
Formulation Components Pip6a–PMO
Size Nm
Zeta Mv
Model Scope in_vivo
Model Type in vivo
Cell Lines Or Primary Cells
Animal Model Severe SMA mouse model (Taiwanese; Smn1−/−; SMN2)
Administration Route systemic intravenous (facial vein / tail vein)
Output Type survival and phenotypic rescue
Output Value median survival up to 456 days; motor and NMJ rescue
Output Units
Output Notes Bodywide SMN restoration including CNS; dose-dependent survival benefit
Toxicity Notes no liver/kidney toxicity; well tolerated
Curation Notes