NBD treatment of animals: Sibling pairs of Ercc1-/delta mice housed in a single cage were treated with 8K-NBD or 8K-mNBD 10 mg/kg three times per week intraperitoneally by an investigator blinded as to the treatment group. Treatment began at 5 weeks of age before the animals were symptomatic and continued throughout their lifespan. Mice were euthanized via CO2 inhalation at 12 or 18-20 weeks of age and tissues isolated. 8K-NBD (KKKKKKKKGGTALDWSWLQTE) and inactive 8K-mutant NBD (8K-mNBD) (KKKKKKKKGGTALDASALQTE) were synthesized at the University of Pittsburgh Peptide facility. Peptides were dissolved in DMSO at 40uM and stored at -80 C until dilution in PBS directly before use.
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