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EXP001488

Paper

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

Peptide

ApoE

Sequence: Ac-LRKLRKRLLRXC-NH2 (X = unspecified spacer as shown in paper schematic)

RNA

splice-switching PMO (morpholino)

All experiment fields

Experiment Id EXP001488
Paper Combination of valproic acid and morpholino splice-switching oligonucleotide produces improved outco
Peptide ApoE
Delivery Success Class no
In Vivo Flag no
Uptake Confirmed no
Label Confidence high
In Vitro Functional Effect yes
Endosomal Escape Evidence
Peptide Concentration 0.5–1 µM (P-PMO)
Rna Concentration 0.5–1 µM (PMO, covalent to peptide)
Mixing Ratio 1:1 (covalent conjugate)
Formulation Format covalent CPP–PMO conjugate
Formulation Components ApoE peptide–PMO (ApoE-PMO)
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: FL-SMN2 and Δ7-SMN2) ± SMN protein (western blot)
Output Value Increased FL-SMN2 and reduced Δ7-SMN2 vs untreated (splice correction in vitro)
Output Units
Output Notes ApoE-PMO alone promoted exon 7 inclusion in SMN2 transcripts (functional splice switching in SMA fibroblasts).
Toxicity Notes No significant toxicity reported for ApoE-PMO at 0.5–1 µM for 4 h or 16 h (MTS assay).
Curation Notes