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Updated: Mar 9, 2026
Assessing Functional Performance in the Mdx Mouse Model
Published on: March 27, 2014
Genetic ablation of P65 subunit of NF-κB in mdx mice to improve muscle physiological function
Xi Yin1,2, Ying Tang1, Jian Li1,3
1Department of Orthopaedic Surgery, University of Pittsburgh, Suite 216, Bridgeside Point II, 450 Technology Drive, Pittsburgh, Pennsylvania, 15219, USA.
Introduction:
Duchenne muscular dystrophy (DMD) is a genetic muscle disease characterized by dystrophin deficiency. Beyond gene replacement, the question of whether ablation of the p65 gene of nuclear factor-kappa B (NF-κB) in DMD can improve muscle physiology function is unknown. In this study, we investigated muscle physiological improvement in mdx mice (DMD model) with a genetic reduction of NF-κB.
Methods:
Muscle physiological function and histology were studied in 2-month-old mdx/p65+/- , wild-type, mdx, and human minidystrophin gene transgenic mdx (TghΔDys/mdx) mice.
Results:
Improved muscle physiological function was found in mdx/p65+/- mice when compared with mdx mice; however, it was similar to TghΔDys/mdx mice. The results indicate that genetic reduction of p65 levels diminished chronic inflammation in dystrophic muscle, thus leading to amelioration of muscle pathology and improved muscle physiological function.
Conclusions:
The results show that inhibition of NF-κB may be a promising therapy when combined with gene therapy for DMD. Muscle Nerve 56: 759-767, 2017.
