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EXP002444

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 EXP002444
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 medium
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 + PGO SSO 187
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 / BTK restoration
Output Value Pip6a-PGO-187 showed activity in reporter cells but minor restoration in primary B cells compared with Pip6a-PGO-186
Output Units
Output Notes Included because it is a CPP-conjugated SSO system tested in reporter and primary B-cell settings
Toxicity Notes Pip6a-PGO-187 sequence updated from supplementary information; lowercase bases are 2′-O-methyl RNA and * indicates PGO/Dmi linkages
Curation Notes