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EXP002435

Paper

DG9-conjugated morpholino rescues phenotype in SMA mice by reaching the CNS via a subcutaneous administration (2023)

Peptide

DG9

Sequence: N-YArVRRrGPRGYArVRRrGPRr-C

RNA

PMO (splice-switching antisense morpholino)

All experiment fields

Experiment Id EXP002435
Paper DG9-conjugated morpholino rescues phenotype in SMA mice by reaching the CNS via a subcutaneous admin
Peptide DG9
Delivery Success Class yes
In Vivo Flag yes
Uptake Confirmed yes
Label Confidence high
In Vitro Functional Effect
Endosomal Escape Evidence
Peptide Concentration
Rna Concentration
Mixing Ratio
Formulation Format covalent peptide–PMO conjugate
Formulation Components DG9–PMO (ISS-N1 targeting PMO)
Size Nm
Zeta Mv
Model Scope in_vivo
Model Type in vivo
Cell Lines Or Primary Cells
Animal Model Severe SMA Taiwanese model: Smn−/−; SMN2Tg/− (Cg-Tg(SMN2)2HungSmn1tm1Hung/J-derived)
Administration Route subcutaneous (postnatal day 0)
Output Type phenotypic rescue / survival; in vivo splice correction
Output Value Median survival: 58 d (40 mg/kg); 121 d (80 mg/kg)
Output Units
Output Notes Single SC dose at PD0; improved weight, motor tests, breathing; increased FL-SMN2 and SMN protein in CNS and peripheral tissues.
Toxicity Notes No apparent toxicity reported up to 80 mg/kg (serum markers, liver/kidney histology, urinary KIM-1); no increase in CD68+ macrophages vs NT.
Curation Notes