title: Genetic ablation of P65 subunit of NF-κB in mdx mice to improve muscle physiological function.
abstract: BACKGROUND
  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.
authors.affiliation: Department of Orthopaedic Surgery, University of Pittsburgh, Suite 216, Bridgeside Point II, 450 Technology Drive, Pittsburgh, Pennsylvania, 15219, USA.|Department of Geriatric Neurology, Chinese PLA General Hospital, Beijing, China.
authors.forename: Xi
authors.initials: X
authors.lastname: Yin
authors.affiliation: Department of Orthopaedic Surgery, University of Pittsburgh, Suite 216, Bridgeside Point II, 450 Technology Drive, Pittsburgh, Pennsylvania, 15219, USA.
authors.forename: Ying
authors.initials: Y
authors.lastname: Tang
authors.affiliation: Department of Orthopaedic Surgery, University of Pittsburgh, Suite 216, Bridgeside Point II, 450 Technology Drive, Pittsburgh, Pennsylvania, 15219, USA.|Beijing Friendship Hospital, Capital Medical University, Beijing, China.
authors.forename: Jian
authors.initials: J
authors.lastname: Li
authors.affiliation: Department of Orthopaedic Surgery, University of Pittsburgh, Suite 216, Bridgeside Point II, 450 Technology Drive, Pittsburgh, Pennsylvania, 15219, USA.
authors.forename: Anna T
authors.initials: AT
authors.lastname: Dzuricky
authors.affiliation: Department of Geriatric Neurology, Chinese PLA General Hospital, Beijing, China.
authors.forename: Chuanqiang
authors.initials: C
authors.lastname: Pu
authors.affiliation: Department of Orthopaedic Surgery, University of Pittsburgh, Suite 216, Bridgeside Point II, 450 Technology Drive, Pittsburgh, Pennsylvania, 15219, USA.
authors.forename: Freddie
authors.initials: F
authors.lastname: Fu
authors.affiliation: Department of Orthopaedic Surgery, University of Pittsburgh, Suite 216, Bridgeside Point II, 450 Technology Drive, Pittsburgh, Pennsylvania, 15219, USA.
authors.forename: Bing
authors.initials: B
authors.lastname: Wang
mesh_terms: D000818:Animals; D017353:Gene Deletion; D006801:Humans; D051379:Mice; D018101:Mice, Inbred mdx; D008822:Mice, Transgenic; D018482:Muscle, Skeletal / Q000473:pathology / Q000502:physiology*; D020388:Muscular Dystrophy, Duchenne / Q000235:genetics* / Q000473:pathology / Q000503:physiopathology; D016328:NF-kappa B / Q000172:deficiency / Q000235:genetics*; D021122:Protein Subunits / Q000172:deficiency / Q000235:genetics*
chemical_list: D016328:NF-kappa B; D021122:Protein Subunits
keywords: DMD; NF-κB pathway; NF-κB/p65 knockout mice; human dystrophin transgenic mice; muscle physiological function