NBD delivery improves the disease phenotype of the golden retriever model of Duchenne muscular dystrophy Article (Faculty180)

cited authors

  • Kornegay, Joe N; Peterson, Jennifer M; Bogan, Daniel J; Kline, William; Bogan, Janet R; Dow, Jennifer L; Fan, Zheng; Wang, Jiahui; Ahn, Mihye; Zhu, Hongtu; Styner, Martin; Guttridge, Denis C

description

  • Duchenne muscular dystrophy (DMD) is caused by mutations in the dystrophin gene and afflicts skeletal and cardiac muscles. Previous studies showed that DMD is associated with constitutive activation of NF-κB, and in dystrophin-deficient mdx and utrophin/dystrophin (utrn (-/-) ;mdx) double knock out (dko) mouse models, inhibition of NF-κB with the Nemo Binding Domain (NBD) peptide led to significant improvements in both diaphragm and cardiac muscle function.

publication date

  • 2014

published in

start page

  • 18

volume

  • 4