In:
Muscle & Nerve, Wiley, Vol. 56, No. 4 ( 2017-10), p. 759-767
Abstract:
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
Type of Medium:
Online Resource
ISSN:
0148-639X
,
1097-4598
Language:
English
Publisher:
Wiley
Publication Date:
2017
detail.hit.zdb_id:
1476641-3
SSG:
12
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