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Long-term effects of systemic gene therapy in a canine model of myotubular myopathy.

Elverman M, Goddard MA, Mack D, Snyder JM, Lawlor MW, Meng H, Beggs AH, Buj-Bello A, Poulard K, Marsh AP, Grange RW, Kelly VE, Childers MK.

Muscle Nerve. 2017 Nov;56(5):943-953. doi: 10.1002/mus.25658. Epub 2017 May 22.


Classical and adaptive control of ex vivo skeletal muscle contractions using Functional Electrical Stimulation (FES).

Jaramillo Cienfuegos P, Shoemaker A, Grange RW, Abaid N, Leonessa A.

PLoS One. 2017 Mar 8;12(3):e0172761. doi: 10.1371/journal.pone.0172761. eCollection 2017.


Systemic AAV8-Mediated Gene Therapy Drives Whole-Body Correction of Myotubular Myopathy in Dogs.

Mack DL, Poulard K, Goddard MA, Latournerie V, Snyder JM, Grange RW, Elverman MR, Denard J, Veron P, Buscara L, Le Bec C, Hogrel JY, Brezovec AG, Meng H, Yang L, Liu F, O'Callaghan M, Gopal N, Kelly VE, Smith BK, Strande JL, Mavilio F, Beggs AH, Mingozzi F, Lawlor MW, Buj-Bello A, Childers MK.

Mol Ther. 2017 Apr 5;25(4):839-854. doi: 10.1016/j.ymthe.2017.02.004. Epub 2017 Feb 22.


Contractile efficiency of dystrophic mdx mouse muscle: in vivo and ex vivo assessment of adaptation to exercise of functional end points.

Capogrosso RF, Mantuano P, Cozzoli A, Sanarica F, Massari AM, Conte E, Fonzino A, Giustino A, Rolland JF, Quaranta A, De Bellis M, Camerino GM, Grange RW, De Luca A.

J Appl Physiol (1985). 2017 Apr 1;122(4):828-843. doi: 10.1152/japplphysiol.00776.2015. Epub 2017 Jan 5.


In Vitro Assays to Determine Skeletal Muscle Physiologic Function.

Sperringer JE, Grange RW.

Methods Mol Biol. 2016;1460:271-91. doi: 10.1007/978-1-4939-3810-0_19.


The SH3 and cysteine-rich domain 3 (Stac3) gene is important to growth, fiber composition, and calcium release from the sarcoplasmic reticulum in postnatal skeletal muscle.

Cong X, Doering J, Mazala DA, Chin ER, Grange RW, Jiang H.

Skelet Muscle. 2016 Apr 11;6:17. doi: 10.1186/s13395-016-0088-4. eCollection 2016.


Dystrophin-deficient dogs with reduced myostatin have unequal muscle growth and greater joint contractures.

Kornegay JN, Bogan DJ, Bogan JR, Dow JL, Wang J, Fan Z, Liu N, Warsing LC, Grange RW, Ahn M, Balog-Alvarez CJ, Cotten SW, Willis MS, Brinkmeyer-Langford C, Zhu H, Palandra J, Morris CA, Styner MA, Wagner KR.

Skelet Muscle. 2016 Apr 4;6:14. doi: 10.1186/s13395-016-0085-7. eCollection 2016.


Muscle pathology, limb strength, walking gait, respiratory function and neurological impairment establish disease progression in the p.N155K canine model of X-linked myotubular myopathy.

Goddard MA, Mack DL, Czerniecki SM, Kelly VE, Snyder JM, Grange RW, Lawlor MW, Smith BK, Beggs AH, Childers MK.

Ann Transl Med. 2015 Oct;3(18):262. doi: 10.3978/j.issn.2305-5839.2015.10.31. Erratum in: Ann Transl Med. 2016 Jan;4(1):23.


Correcting Neuromuscular Deficits With Gene Therapy in Pompe Disease.

Todd AG, McElroy JA, Grange RW, Fuller DD, Walter GA, Byrne BJ, Falk DJ.

Ann Neurol. 2015 Aug;78(2):222-34. doi: 10.1002/ana.24433. Epub 2015 Jun 30.


C57BL/6 life span study: age-related declines in muscle power production and contractile velocity.

Graber TG, Kim JH, Grange RW, McLoon LK, Thompson LV.

Age (Dordr). 2015 Jun;37(3):9773. doi: 10.1007/s11357-015-9773-1. Epub 2015 Apr 17.


Nutrients and muscle disease.

Nisoli E, Grange RW, D'Antona G.

Biomed Res Int. 2015;2015:809830. doi: 10.1155/2015/809830. Epub 2015 Mar 19. No abstract available.


The role of proteases in excitation-contraction coupling failure in muscular dystrophy.

Mázala DA, Grange RW, Chin ER.

Am J Physiol Cell Physiol. 2015 Jan 1;308(1):C33-40. doi: 10.1152/ajpcell.00267.2013. Epub 2014 Oct 8.


Tissue triage and freezing for models of skeletal muscle disease.

Meng H, Janssen PM, Grange RW, Yang L, Beggs AH, Swanson LC, Cossette SA, Frase A, Childers MK, Granzier H, Gussoni E, Lawlor MW.

J Vis Exp. 2014 Jul 15;(89). doi: 10.3791/51586.


A mathematical model of skeletal muscle disease and immune response in the mdx mouse.

Jarrah AS, Castiglione F, Evans NP, Grange RW, Laubenbacher R.

Biomed Res Int. 2014;2014:871810. doi: 10.1155/2014/871810. Epub 2014 Jun 11.


KLHL40 deficiency destabilizes thin filament proteins and promotes nemaline myopathy.

Garg A, O'Rourke J, Long C, Doering J, Ravenscroft G, Bezprozvannaya S, Nelson BR, Beetz N, Li L, Chen S, Laing NG, Grange RW, Bassel-Duby R, Olson EN.

J Clin Invest. 2014 Aug;124(8):3529-39. doi: 10.1172/JCI74994. Epub 2014 Jun 24.


Compared with that of MUFA, a high dietary intake of n-3 PUFA does not reduce the degree of pathology in mdx mice.

Henderson GC, Evans NP, Grange RW, Tuazon MA.

Br J Nutr. 2014 May 28;111(10):1791-800. doi: 10.1017/S0007114514000129. Epub 2014 Feb 13.


Gene therapy prolongs survival and restores function in murine and canine models of myotubular myopathy.

Childers MK, Joubert R, Poulard K, Moal C, Grange RW, Doering JA, Lawlor MW, Rider BE, Jamet T, Danièle N, Martin S, Rivière C, Soker T, Hammer C, Van Wittenberghe L, Lockard M, Guan X, Goddard M, Mitchell E, Barber J, Williams JK, Mack DL, Furth ME, Vignaud A, Masurier C, Mavilio F, Moullier P, Beggs AH, Buj-Bello A.

Sci Transl Med. 2014 Jan 22;6(220):220ra10. doi: 10.1126/scitranslmed.3007523.


Myosin phosphorylation and force potentiation in skeletal muscle: evidence from animal models.

Vandenboom R, Gittings W, Smith IC, Grange RW, Stull JT.

J Muscle Res Cell Motil. 2013 Dec;34(5-6):317-32. doi: 10.1007/s10974-013-9363-8. Epub 2013 Oct 27. Review.


Selenoprotein N deficiency in mice is associated with abnormal lung development.

Moghadaszadeh B, Rider BE, Lawlor MW, Childers MK, Grange RW, Gupta K, Boukedes SS, Owen CA, Beggs AH.

FASEB J. 2013 Apr;27(4):1585-99. doi: 10.1096/fj.12-212688. Epub 2013 Jan 16.


Enzyme replacement therapy rescues weakness and improves muscle pathology in mice with X-linked myotubular myopathy.

Lawlor MW, Armstrong D, Viola MG, Widrick JJ, Meng H, Grange RW, Childers MK, Hsu CP, O'Callaghan M, Pierson CR, Buj-Bello A, Beggs AH.

Hum Mol Genet. 2013 Apr 15;22(8):1525-38. doi: 10.1093/hmg/ddt003. Epub 2013 Jan 9.


Muscle function in a canine model of X-linked myotubular myopathy.

Grange RW, Doering J, Mitchell E, Holder MN, Guan X, Goddard M, Tegeler C, Beggs AH, Childers MK.

Muscle Nerve. 2012 Oct;46(4):588-91. doi: 10.1002/mus.23463.


Foxk1 promotes cell proliferation and represses myogenic differentiation by regulating Foxo4 and Mef2.

Shi X, Wallis AM, Gerard RD, Voelker KA, Grange RW, DePinho RA, Garry MG, Garry DJ.

J Cell Sci. 2012 Nov 15;125(Pt 22):5329-37. doi: 10.1242/jcs.105239. Epub 2012 Sep 6.


Exercise and Duchenne muscular dystrophy: where we have been and where we need to go.

Markert CD, Case LE, Carter GT, Furlong PA, Grange RW.

Muscle Nerve. 2012 May;45(5):746-51. doi: 10.1002/mus.23244. No abstract available.


Chronic administration of a leupeptin-derived calpain inhibitor fails to ameliorate severe muscle pathology in a canine model of duchenne muscular dystrophy.

Childers MK, Bogan JR, Bogan DJ, Greiner H, Holder M, Grange RW, Kornegay JN.

Front Pharmacol. 2012 Jan 9;2:89. doi: 10.3389/fphar.2011.00089. eCollection 2011.


Nutrition strategies to improve physical capabilities in Duchenne muscular dystrophy.

Davoodi J, Markert CD, Voelker KA, Hutson SM, Grange RW.

Phys Med Rehabil Clin N Am. 2012 Feb;23(1):187-99, xii-xiii. doi: 10.1016/j.pmr.2011.11.010. Epub 2011 Dec 13. Review.


The paradox of muscle hypertrophy in muscular dystrophy.

Kornegay JN, Childers MK, Bogan DJ, Bogan JR, Nghiem P, Wang J, Fan Z, Howard JF Jr, Schatzberg SJ, Dow JL, Grange RW, Styner MA, Hoffman EP, Wagner KR.

Phys Med Rehabil Clin N Am. 2012 Feb;23(1):149-72, xii. doi: 10.1016/j.pmr.2011.11.014. Review.


Canine models of Duchenne muscular dystrophy and their use in therapeutic strategies.

Kornegay JN, Bogan JR, Bogan DJ, Childers MK, Li J, Nghiem P, Detwiler DA, Larsen CA, Grange RW, Bhavaraju-Sanka RK, Tou S, Keene BP, Howard JF Jr, Wang J, Fan Z, Schatzberg SJ, Styner MA, Flanigan KM, Xiao X, Hoffman EP.

Mamm Genome. 2012 Feb;23(1-2):85-108. doi: 10.1007/s00335-011-9382-y. Epub 2012 Jan 5. Review.


Aging and physical mobility in group-housed Old World monkeys.

Shively CA, Willard SL, Register TC, Bennett AJ, Pierre PJ, Laudenslager ML, Kitzman DW, Childers MK, Grange RW, Kritchevsky SB.

Age (Dordr). 2012 Oct;34(5):1123-31. Epub 2011 Dec 28.


Mice lacking microRNA 133a develop dynamin 2–dependent centronuclear myopathy.

Liu N, Bezprozvannaya S, Shelton JM, Frisard MI, Hulver MW, McMillan RP, Wu Y, Voelker KA, Grange RW, Richardson JA, Bassel-Duby R, Olson EN.

J Clin Invest. 2011 Aug;121(8):3258-68. doi: 10.1172/JCI46267.


Concerted regulation of myofiber-specific gene expression and muscle performance by the transcriptional repressor Sox6.

Quiat D, Voelker KA, Pei J, Grishin NV, Grange RW, Bassel-Duby R, Olson EN.

Proc Natl Acad Sci U S A. 2011 Jun 21;108(25):10196-201. doi: 10.1073/pnas.1107413108. Epub 2011 Jun 1.


Dietary epicatechin promotes survival of obese diabetic mice and Drosophila melanogaster.

Si H, Fu Z, Babu PV, Zhen W, Leroith T, Meaney MP, Voelker KA, Jia Z, Grange RW, Liu D.

J Nutr. 2011 Jun;141(6):1095-100. doi: 10.3945/jn.110.134270. Epub 2011 Apr 27.


Myosin light-chain phosphorylation and potentiation of dynamic function in mouse fast muscle.

Xeni J, Gittings WB, Caterini D, Huang J, Houston ME, Grange RW, Vandenboom R.

Pflugers Arch. 2011 Aug;462(2):349-58. doi: 10.1007/s00424-011-0965-y. Epub 2011 Apr 16.


In vivo canine muscle function assay.

Childers MK, Grange RW, Kornegay JN.

J Vis Exp. 2011 Apr 5;(50). pii: 2623. doi: 10.3791/2623.


The passive mechanical properties of the extensor digitorum longus muscle are compromised in 2- to 20-mo-old mdx mice.

Hakim CH, Grange RW, Duan D.

J Appl Physiol (1985). 2011 Jun;110(6):1656-63. doi: 10.1152/japplphysiol.01425.2010. Epub 2011 Mar 17.


Exercise and Duchenne muscular dystrophy: toward evidence-based exercise prescription.

Markert CD, Ambrosio F, Call JA, Grange RW.

Muscle Nerve. 2011 Apr;43(4):464-78. doi: 10.1002/mus.21987. Review.


Golden retriever muscular dystrophy (GRMD): Developing and maintaining a colony and physiological functional measurements.

Kornegay JN, Bogan JR, Bogan DJ, Childers MK, Grange RW.

Methods Mol Biol. 2011;709:105-23. doi: 10.1007/978-1-61737-982-6_7.


Eccentric contractions induce rapid isometric torque drop in dystrophin-deficient dogs.

Tegeler CJ, Grange RW, Bogan DJ, Markert CD, Case D, Kornegay JN, Childers MK.

Muscle Nerve. 2010 Jul;42(1):130-2. doi: 10.1002/mus.21699.


Functional muscle analysis of the Tcap knockout mouse.

Markert CD, Meaney MP, Voelker KA, Grange RW, Dalley HW, Cann JK, Ahmed M, Bishwokarma B, Walker SJ, Yu SX, Brown M, Lawlor MW, Beggs AH, Childers MK.

Hum Mol Genet. 2010 Jun 1;19(11):2268-83. doi: 10.1093/hmg/ddq105. Epub 2010 Mar 16.


Foxj3 transcriptionally activates Mef2c and regulates adult skeletal muscle fiber type identity.

Alexander MS, Shi X, Voelker KA, Grange RW, Garcia JA, Hammer RE, Garry DJ.

Dev Biol. 2010 Jan 15;337(2):396-404. doi: 10.1016/j.ydbio.2009.11.015. Epub 2009 Nov 13.


Green tea extract decreases muscle pathology and NF-kappaB immunostaining in regenerating muscle fibers of mdx mice.

Evans NP, Call JA, Bassaganya-Riera J, Robertson JL, Grange RW.

Clin Nutr. 2010 Jun;29(3):391-8. doi: 10.1016/j.clnu.2009.10.001. Epub 2009 Nov 7.


Immune-mediated mechanisms potentially regulate the disease time-course of duchenne muscular dystrophy and provide targets for therapeutic intervention.

Evans NP, Misyak SA, Robertson JL, Bassaganya-Riera J, Grange RW.

PM R. 2009 Aug;1(8):755-68. doi: 10.1016/j.pmrj.2009.04.010. Review.


The F-BAR protein CIP4 promotes GLUT4 endocytosis through bidirectional interactions with N-WASp and Dynamin-2.

Hartig SM, Ishikura S, Hicklen RS, Feng Y, Blanchard EG, Voelker KA, Pichot CS, Grange RW, Raphael RM, Klip A, Corey SJ.

J Cell Sci. 2009 Jul 1;122(Pt 13):2283-91. doi: 10.1242/jcs.041343. Epub 2009 Jun 9.


Dysregulated intracellular signaling and inflammatory gene expression during initial disease onset in Duchenne muscular dystrophy.

Evans NP, Misyak SA, Robertson JL, Bassaganya-Riera J, Grange RW.

Am J Phys Med Rehabil. 2009 Jun;88(6):502-22. doi: 10.1097/PHM.0b013e3181a5a24f. Review.


Endurance capacity in maturing mdx mice is markedly enhanced by combined voluntary wheel running and green tea extract.

Call JA, Voelker KA, Wolff AV, McMillan RP, Evans NP, Hulver MW, Talmadge RJ, Grange RW.

J Appl Physiol (1985). 2008 Sep;105(3):923-32. doi: 10.1152/japplphysiol.00028.2008. Epub 2008 Jun 26.


A novel SNaPshot assay to detect the mdx mutation.

Budowle SA, Gonzalez S, Budowle B, Eisenberg AJ, Grange RW.

Muscle Nerve. 2008 Jun;37(6):731-5. doi: 10.1002/mus.21027.


Telomere shortening in diaphragm and tibialis anterior muscles of aged mdx mice.

Lund TC, Grange RW, Lowe DA.

Muscle Nerve. 2007 Sep;36(3):387-90.


Recommendations to define exercise prescription for Duchenne muscular dystrophy.

Grange RW, Call JA.

Exerc Sport Sci Rev. 2007 Jan;35(1):12-7. Review.


Passive mechanical properties of maturing extensor digitorum longus are not affected by lack of dystrophin.

Wolff AV, Niday AK, Voelker KA, Call JA, Evans NP, Granata KP, Grange RW.

Muscle Nerve. 2006 Sep;34(3):304-12.


Increased extraocular muscle strength with direct injection of insulin-like growth factor-I.

Anderson BC, Christiansen SP, Grandt S, Grange RW, McLoon LK.

Invest Ophthalmol Vis Sci. 2006 Jun;47(6):2461-7.

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