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EXP002441

Paper

Cell-penetrating peptide-conjugated, splice-switching oligonucleotides mitigate the phenotype in Btk/Tec double deficient X-linked agammaglobulinemia model (2025)

Peptide

Pip6a-PMO 186

Sequence: Ac-RXRRBRRXRYQFLIRXRBRXRB (Pip6a; X = 6-aminohexanoic acid, B = beta-alanine)

RNA

SCO

All experiment fields

Experiment Id EXP002441
Paper Cell-penetrating peptide-conjugated, splice-switching oligonucleotides mitigate the phenotype in Btk
Peptide Pip6a-PMO 186
Delivery Success Class no
In Vivo Flag no
Uptake Confirmed no
Label Confidence high
In Vitro Functional Effect yes
Endosomal Escape Evidence
Peptide Concentration 0.625–5 µM in reporter-cell assay; 0.3 and 5 µM in primary B-cell ex vivo assay
Rna Concentration
Mixing Ratio
Formulation Format covalent peptide-oligonucleotide conjugate
Formulation Components Pip6a cell-penetrating peptide + PMO SSO 186
Size Nm
Zeta Mv
Model Scope ex_vivo
Model Type in vitro / ex vivo
Cell Lines Or Primary Cells U2OS luciferase BTK intron 4 reporter cells; primary B cells from BAC transgenic mouse
Animal Model
Administration Route cell incubation
Output Type splice correction and BTK protein restoration
Output Value Reporter cells: strong luciferase rescue and corrected BTK RNA; primary B cells: detectable BTK protein after 48 h
Output Units
Output Notes Pip6a-PMO 186 had superior activity over B-PMO 186 and was one of the most efficient ex vivo conjugates
Toxicity Notes Reporter and primary B-cell data; exact Pip6a and PMO 186 sequences updated from supplementary information
Curation Notes