Back to browse

EXP001685

Paper

Peptide-conjugated oligonucleotides evoke long-lasting myotonic dystrophy correction in patient-derived cells and mice (2019)

Peptide

Pip6a-PMO-CAG7

Sequence: Not reported in main text (Pip6a described as hydrophobic core flanked by arginine-rich domains with β-alanine and aminohexanoyl spacers).

RNA

PMO (morpholino antisense oligonucleotide)

All experiment fields

Experiment Id EXP001685
Paper Peptide-conjugated oligonucleotides evoke long-lasting myotonic dystrophy correction in patient-deri
Peptide Pip6a-PMO-CAG7
Delivery Success Class no
In Vivo Flag no
Uptake Confirmed no
Label Confidence high
In Vitro Functional Effect yes
Endosomal Escape Evidence
Peptide Concentration
Rna Concentration
Mixing Ratio Covalent conjugate (1:1 peptide:PMO)
Formulation Format Peptide–PMO conjugate (Pip6a-PMO)
Formulation Components Pip6a peptide covalently conjugated to PMO-CAG7 (or Pip6a-Ctrl control sequence)
Size Nm
Zeta Mv
Model Scope in_vitro
Model Type in vitro
Cell Lines Or Primary Cells Patient-derived DM1 differentiated myoblasts (immortalized; 2600 CTG; also 1300 CTG line in supplemental)
Animal Model
Administration Route
Output Type in vitro splice correction / RNA foci reduction
Output Value Significant correction of MBNL1-dependent splicing defects (e.g., LDB3, MBNL1, SOS1, DMD) and reduction of nuclear RNA foci; decreased mutant DMPK transcript levels in DM1 myoblasts after 24 h.
Output Units
Output Notes Differentiated immortalized DM1 myoblasts (2600 CTG repeats) treated with 1 µM Pip6a-PMO for 24 h; FISH/immunofluorescence shows MBNL1 relocation and fewer CUGexp RNA foci; RT-PCR shows splice correction; Northern blot shows reduced mutant DMPK.
Toxicity Notes
Curation Notes