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Peptide-based inhibition of NF-κB rescues diaphragm muscle contractile dysfunction in a murine model of Duchenne muscular dystrophy.

Peterson JM, Kline W, Canan BD, Ricca DJ, Kaspar B, Delfín DA, DiRienzo K, Clemens PR, Robbins PD, Baldwin AS, Flood P, Kaumaya P, Freitas M, Kornegay JN, Mendell JR, Rafael-Fortney JA, Guttridge DC, Janssen PM.

Mol Med. 2011 May-Jun;17(5-6):508-15. doi: 10.2119/molmed.2010.00263. Epub 2011 Jan 20.


Systemic delivery of NEMO binding domain/IKKγ inhibitory peptide to young mdx mice improves dystrophic skeletal muscle histopathology.

Reay DP, Yang M, Watchko JF, Daood M, O'Day TL, Rehman KK, Guttridge DC, Robbins PD, Clemens PR.

Neurobiol Dis. 2011 Sep;43(3):598-608. doi: 10.1016/j.nbd.2011.05.008. Epub 2011 May 23.


Improvement of cardiac contractile function by peptide-based inhibition of NF-κB in the utrophin/dystrophin-deficient murine model of muscular dystrophy.

Delfín DA, Xu Y, Peterson JM, Guttridge DC, Rafael-Fortney JA, Janssen PM.

J Transl Med. 2011 May 17;9:68. doi: 10.1186/1479-5876-9-68.


Effect of nuclear factor κB inhibition on serotype 9 adeno-associated viral (AAV9) minidystrophin gene transfer to the mdx mouse.

Reay DP, Niizawa GA, Watchko JF, Daood M, Reay JC, Raggi E, Clemens PR.

Mol Med. 2012 May 9;18:466-76. doi: 10.2119/molmed.2011.00404.


Sildenafil reduces respiratory muscle weakness and fibrosis in the mdx mouse model of Duchenne muscular dystrophy.

Percival JM, Whitehead NP, Adams ME, Adamo CM, Beavo JA, Froehner SC.

J Pathol. 2012 Sep;228(1):77-87. doi: 10.1002/path.4054. Epub 2012 Jul 18.


D-Amino Acid Substitution of Peptide-Mediated NF-κB Suppression in mdx Mice Preserves Therapeutic Benefit in Skeletal Muscle, but Causes Kidney Toxicity.

Reay DP, Bastacky SI, Wack KE, Stolz DB, Robbins PD, Clemens PR.

Mol Med. 2015 May 22;21:442-52. doi: 10.2119/molmed.2013.00141.


Progressive nuclear factor-kappaB activation resistant to inhibition by contraction and curcumin in mdx mice.

Durham WJ, Arbogast S, Gerken E, Li YP, Reid MB.

Muscle Nerve. 2006 Sep;34(3):298-303.


Force and power output of diaphragm muscle strips from mdx and control mice after clenbuterol treatment.

Lynch GS, Hinkle RT, Faulkner JA.

Neuromuscul Disord. 2001 Mar;11(2):192-6.


Pentoxifylline fails to attenuate fibrosis in dystrophic (mdx) diaphragm muscle.

Gosselin LE, Williams JE.

Muscle Nerve. 2006 Jun;33(6):820-3.


Contribution of oxidative stress to pathology in diaphragm and limb muscles with Duchenne muscular dystrophy.

Kim JH, Kwak HB, Thompson LV, Lawler JM.

J Muscle Res Cell Motil. 2013 Feb;34(1):1-13. doi: 10.1007/s10974-012-9330-9. Epub 2012 Oct 28. Review.


Deubiquitinating enzyme A20 negatively regulates NF-κB signaling in skeletal muscle in mdx mice.

Charan RA, Hanson R, Clemens PR.

FASEB J. 2012 Feb;26(2):587-95. doi: 10.1096/fj.11-189829. Epub 2011 Oct 19. Erratum in: FASEB J. 2013 Jan;27(1):410.


Improved contractile function of the mdx dystrophic mouse diaphragm muscle after insulin-like growth factor-I administration.

Gregorevic P, Plant DR, Leeding KS, Bach LA, Lynch GS.

Am J Pathol. 2002 Dec;161(6):2263-72.


Long-Term Therapy With Omega-3 Ameliorates Myonecrosis and Benefits Skeletal Muscle Regeneration in Mdx Mice.

Apolinário LM, De Carvalho SC, Santo Neto H, Marques MJ.

Anat Rec (Hoboken). 2015 Sep;298(9):1589-96. doi: 10.1002/ar.23177. Epub 2015 Jun 22.


Loss of cIAP1 attenuates soleus muscle pathology and improves diaphragm function in mdx mice.

Enwere EK, Boudreault L, Holbrook J, Timusk K, Earl N, LaCasse E, Renaud JM, Korneluk RG.

Hum Mol Genet. 2013 Mar 1;22(5):867-78. doi: 10.1093/hmg/dds493. Epub 2012 Nov 25.


Reduction of Oxidative Damage and Inflammatory Response in the Diaphragm Muscle of mdx Mice Using Iron Chelator Deferoxamine.

Moraes LH, de Burgos RR, Macedo AB, de Almeida Hermes T, de Faria FM, Minatel E.

Biol Trace Elem Res. 2015 Sep;167(1):115-20. doi: 10.1007/s12011-015-0290-y. Epub 2015 Mar 13.


Excessive collagen accumulation in dystrophic (mdx) respiratory musculature is independent of enhanced activation of the NF-kappaB pathway.

Graham KM, Singh R, Millman G, Malnassy G, Gatti F, Bruemmer K, Stefanski C, Curtis H, Sesti J, Carlson CG.

J Neurol Sci. 2010 Jul 15;294(1-2):43-50. doi: 10.1016/j.jns.2010.04.007. Epub 2010 May 13.


Inhibition of the IKK/NF-κB pathway by AAV gene transfer improves muscle regeneration in older mdx mice.

Tang Y, Reay DP, Salay MN, Mi MY, Clemens PR, Guttridge DC, Robbins PD, Huard J, Wang B.

Gene Ther. 2010 Dec;17(12):1476-83. doi: 10.1038/gt.2010.110. Epub 2010 Aug 19.


Nuclear factor kappa-B blockade reduces skeletal muscle degeneration and enhances muscle function in Mdx mice.

Messina S, Bitto A, Aguennouz M, Minutoli L, Monici MC, Altavilla D, Squadrito F, Vita G.

Exp Neurol. 2006 Mar;198(1):234-41. Epub 2006 Jan 10.


Treatment with inhibitors of the NF-kappaB pathway improves whole body tension development in the mdx mouse.

Siegel AL, Bledsoe C, Lavin J, Gatti F, Berge J, Millman G, Turin E, Winders WT, Rutter J, Palmeiri B, Carlson CG.

Neuromuscul Disord. 2009 Feb;19(2):131-9. doi: 10.1016/j.nmd.2008.10.006. Epub 2008 Dec 2.

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