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EXP001489

Paper

Combination of valproic acid and morpholino splice-switching oligonucleotide produces improved outcomes in spinal muscular atrophy patient-derived fibroblasts (2017)

Peptide

(N)-VPA-ApoE

Sequence: VPA–XLRKLRKRLLRXC-NH2 (as shown in paper schematic)

RNA

splice-switching PMO (morpholino)

All experiment fields

Experiment Id EXP001489
Paper Combination of valproic acid and morpholino splice-switching oligonucleotide produces improved outco
Peptide (N)-VPA-ApoE
Delivery Success Class no
In Vivo Flag no
Uptake Confirmed no
Label Confidence high
In Vitro Functional Effect no
Endosomal Escape Evidence
Peptide Concentration 0.5–1 µM (P-PMO) (toxicity tested up to 4 µM)
Rna Concentration 0.5–1 µM (PMO, covalent to peptide)
Mixing Ratio 1:1 (covalent conjugate)
Formulation Format covalent VPA–CPP–PMO conjugate
Formulation Components (N)-VPA-ApoE-PMO (amide-linked VPA)
Size Nm
Zeta Mv
Model Scope in_vitro
Model Type in vitro
Cell Lines Or Primary Cells SMA Type I patient-derived fibroblasts (GM03813, Coriell)
Animal Model
Administration Route
Output Type SMN2 splice switching (RT-qPCR)
Output Value Slight increase in FL-SMN2 (not statistically significant vs untreated)
Output Units
Output Notes (N)-VPA-ApoE-PMO showed poor efficacy vs VPA + ApoE-PMO co-treatment; splice correction not clearly demonstrated beyond noise.
Toxicity Notes At 16 h, 4 µM (N)-VPA-ApoE-PMO showed significant cytotoxicity (reported cell viability ~72%).
Curation Notes