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Inhibiting Bruton's tyrosine kinase rescues mice from lethal influenza-induced acute lung injury.

Florence JM, Krupa A, Booshehri LM, Davis SA, Matthay MA, Kurdowska AK.

Am J Physiol Lung Cell Mol Physiol. 2018 Jul 1;315(1):L52-L58. doi: 10.1152/ajplung.00047.2018. Epub 2018 Mar 8.


Disrupting the Btk Pathway Suppresses COPD-Like Lung Alterations in Atherosclerosis Prone ApoE-/- Mice Following Regular Exposure to Cigarette Smoke.

Florence JM, Krupa A, Booshehri LM, Gajewski AL, Kurdowska AK.

Int J Mol Sci. 2018 Jan 24;19(2). pii: E343. doi: 10.3390/ijms19020343.


Characterization of Strength and Function in Ambulatory Adults With GNE Myopathy.

Argov Z, Bronstein F, Esposito A, Feinsod-Meiri Y, Florence JM, Fowler E, Greenberg MB, Malkus EC, Rebibo O, Siener CS, Caraco Y, Kolodny EH, Lau HA, Pestronk A, Shieh P, Skrinar AM, Mayhew JE.

J Clin Neuromuscul Dis. 2017 Sep;19(1):19-26. doi: 10.1097/CND.0000000000000181.


Metalloproteinase-9 contributes to endothelial dysfunction in atherosclerosis via protease activated receptor-1.

Florence JM, Krupa A, Booshehri LM, Allen TC, Kurdowska AK.

PLoS One. 2017 Feb 6;12(2):e0171427. doi: 10.1371/journal.pone.0171427. eCollection 2017.


Efficacy and safety of deflazacort vs prednisone and placebo for Duchenne muscular dystrophy.

Griggs RC, Miller JP, Greenberg CR, Fehlings DL, Pestronk A, Mendell JR, Moxley RT 3rd, King W, Kissel JT, Cwik V, Vanasse M, Florence JM, Pandya S, Dubow JS, Meyer JM.

Neurology. 2016 Nov 15;87(20):2123-2131. Epub 2016 Aug 26.


Clinical trial readiness in non-ambulatory boys and men with duchenne muscular dystrophy: MDA-DMD network follow-up.

Connolly AM, Florence JM, Zaidman CM, Golumbek PT, Mendell JR, Flanigan KM, Karachunski PI, Day JW, McDonald CM, Darras BT, Kang PB, Siener CA, Gadeken RK, Anand P, Schierbecker JR, Malkus EC, Lowes LP, Alfano LN, Johnson L, Nicorici A, Kelecic JM, Quigley J, Pasternak AE, Miller JP; MDA-DMD Clinical Research Network.

Muscle Nerve. 2016 Oct;54(4):681-9. doi: 10.1002/mus.25089. Epub 2016 May 10.


Clinical phenotypes as predictors of the outcome of skipping around DMD exon 45.

Findlay AR, Wein N, Kaminoh Y, Taylor LE, Dunn DM, Mendell JR, King WM, Pestronk A, Florence JM, Mathews KD, Finkel RS, Swoboda KJ, Howard MT, Day JW, McDonald C, Nicolas A, Le Rumeur E, Weiss RB, Flanigan KM; United Dystrophinopathy Project.

Ann Neurol. 2015 Apr;77(4):668-74. doi: 10.1002/ana.24365. Epub 2015 Mar 2.


Silencing Bruton's tyrosine kinase in alveolar neutrophils protects mice from LPS/immune complex-induced acute lung injury.

Krupa A, Fol M, Rahman M, Stokes KY, Florence JM, Leskov IL, Khoretonenko MV, Matthay MA, Liu KD, Calfee CS, Tvinnereim A, Rosenfield GR, Kurdowska AK.

Am J Physiol Lung Cell Mol Physiol. 2014 Sep 15;307(6):L435-48. doi: 10.1152/ajplung.00234.2013. Epub 2014 Aug 1.


Outcome reliability in non-ambulatory boys/men with Duchenne muscular dystrophy.

Connolly AM, Malkus EC, Mendell JR, Flanigan KM, Miller JP, Schierbecker JR, Siener CA, Golumbek PT, Zaidman CM, Mcdonald CM, Johnson L, Nicorici A, Karachunski PI, Day JW, Kelecic JM, Lowes LP, Alfano LN, Darras BT, Kang PB, Quigley J, Pasternak AE, Florence JM; MDA DMD Clinical Research Network.

Muscle Nerve. 2015 Apr;51(4):522-32. doi: 10.1002/mus.24346. Epub 2015 Feb 11.


One year outcome of boys with Duchenne muscular dystrophy using the Bayley-III scales of infant and toddler development.

Connolly AM, Florence JM, Cradock MM, Eagle M, Flanigan KM, McDonald CM, Karachunski PI, Darras BT, Bushby K, Malkus EC, Golumbek PT, Zaidman CM, Miller JP, Mendell JR; MDA DMD Clinical Research Network.

Pediatr Neurol. 2014 Jun;50(6):557-63. doi: 10.1016/j.pediatrneurol.2014.02.006. Epub 2014 Feb 15.


Therapeutic exercise for people with amyotrophic lateral sclerosis or motor neuron disease.

Dal Bello-Haas V, Florence JM.

Cochrane Database Syst Rev. 2013 May 31;(5):CD005229. doi: 10.1002/14651858.CD005229.pub3. Review.


Motor and cognitive assessment of infants and young boys with Duchenne Muscular Dystrophy: results from the Muscular Dystrophy Association DMD Clinical Research Network.

Connolly AM, Florence JM, Cradock MM, Malkus EC, Schierbecker JR, Siener CA, Wulf CO, Anand P, Golumbek PT, Zaidman CM, Philip Miller J, Lowes LP, Alfano LN, Viollet-Callendret L, Flanigan KM, Mendell JR, McDonald CM, Goude E, Johnson L, Nicorici A, Karachunski PI, Day JW, Dalton JC, Farber JM, Buser KK, Darras BT, Kang PB, Riley SO, Shriber E, Parad R, Bushby K, Eagle M; MDA DMD Clinical Research Network.

Neuromuscul Disord. 2013 Jul;23(7):529-39. doi: 10.1016/j.nmd.2013.04.005. Epub 2013 May 28.


The 6-minute walk test and other endpoints in Duchenne muscular dystrophy: longitudinal natural history observations over 48 weeks from a multicenter study.

McDonald CM, Henricson EK, Abresch RT, Florence JM, Eagle M, Gappmaier E, Glanzman AM; PTC124-GD-007-DMD Study Group, Spiegel R, Barth J, Elfring G, Reha A, Peltz S.

Muscle Nerve. 2013 Sep;48(3):343-56. doi: 10.1002/mus.23902. Epub 2013 Jun 26.


The cooperative international neuromuscular research group Duchenne natural history study--a longitudinal investigation in the era of glucocorticoid therapy: design of protocol and the methods used.

McDonald CM, Henricson EK, Abresch RT, Han JJ, Escolar DM, Florence JM, Duong T, Arrieta A, Clemens PR, Hoffman EP, Cnaan A; Cinrg Investigators.

Muscle Nerve. 2013 Jul;48(1):32-54. doi: 10.1002/mus.23807. Epub 2013 May 16.


The cooperative international neuromuscular research group Duchenne natural history study: glucocorticoid treatment preserves clinically meaningful functional milestones and reduces rate of disease progression as measured by manual muscle testing and other commonly used clinical trial outcome measures.

Henricson EK, Abresch RT, Cnaan A, Hu F, Duong T, Arrieta A, Han J, Escolar DM, Florence JM, Clemens PR, Hoffman EP, McDonald CM; CINRG Investigators.

Muscle Nerve. 2013 Jul;48(1):55-67. doi: 10.1002/mus.23808. Epub 2013 May 6.


LTBP4 genotype predicts age of ambulatory loss in Duchenne muscular dystrophy.

Flanigan KM, Ceco E, Lamar KM, Kaminoh Y, Dunn DM, Mendell JR, King WM, Pestronk A, Florence JM, Mathews KD, Finkel RS, Swoboda KJ, Gappmaier E, Howard MT, Day JW, McDonald C, McNally EM, Weiss RB; United Dystrophinopathy Project.

Ann Neurol. 2013 Apr;73(4):481-8. doi: 10.1002/ana.23819. Epub 2013 Feb 20.


Bruton's tyrosine kinase mediates FcγRIIa/Toll-like receptor-4 receptor crosstalk in human neutrophils.

Krupa A, Fudala R, Florence JM, Tucker T, Allen TC, Standiford TJ, Luchowski R, Fol M, Rahman M, Gryczynski Z, Gryczynski I, Kurdowska AK.

Am J Respir Cell Mol Biol. 2013 Feb;48(2):240-9. doi: 10.1165/rcmb.2012-0039OC. Epub 2012 Dec 13.


Fas-deficient mice have impaired alveolar neutrophil recruitment and decreased expression of anti-KC autoantibody:KC complexes in a model of acute lung injury.

Gil S, Farnand AW, Altemeier WA, Gill SE, Kurdowska A, Krupa A, Florence JM, Matute-Bello G.

Respir Res. 2012 Oct 9;13:91. doi: 10.1186/1465-9921-13-91.


Physical therapy evaluation and management in neuromuscular diseases.

Johnson LB, Florence JM, Abresch RT.

Phys Med Rehabil Clin N Am. 2012 Aug;23(3):633-51. doi: 10.1016/j.pmr.2012.06.005. Review.


Consensus statement on standard of care for congenital myopathies.

Wang CH, Dowling JJ, North K, Schroth MK, Sejersen T, Shapiro F, Bellini J, Weiss H, Guillet M, Amburgey K, Apkon S, Bertini E, Bonnemann C, Clarke N, Connolly AM, Estournet-Mathiaud B, Fitzgerald D, Florence JM, Gee R, Gurgel-Giannetti J, Glanzman AM, Hofmeister B, Jungbluth H, Koumbourlis AC, Laing NG, Main M, Morrison LA, Munns C, Rose K, Schuler PM, Sewry C, Storhaug K, Vainzof M, Yuan N.

J Child Neurol. 2012 Mar;27(3):363-82. doi: 10.1177/0883073812436605.

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