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Arthur Burghes , Ph.D.
Professor

The Ohio State University
1645 Neil Avenue
Columbus, OH 43210

Phone: (614) 688-4579
Fax: (614) 292-4118
Email: burghes.1@osu.edu

Education & Training:
Hammersmith Hospital, University London, Ph.D.
Hospital for Sick Children, University of Toronto, Postdoctoral Fellow

Selected Publications:

• Burghes AH, McGovern VL. (2010) “ Antisense oligonucleotides and spinal muscular atrophy: skipping along” Genes Dev. 24:1574-9.

• Foust KD, Wang X, McGovern VL, Braun L, Bevan AK, Haidet AM, Le TT, Morales PR, Rich MM, Burghes AH, Kaspar BK. (2010) “ Rescue of the spinal muscular atrophy phenotype in a mouse model by early postnatal delivery of SMN” Nat Biotechnol. 28:271-4.

• Butchbach ME, Singh J, Thorsteinsdóttir M, Saieva L, Slominski E, Thurmond J, Andrésson T, Zhang J, Edwards JD, Simard LR, Pellizzoni L, Jarecki J, Burghes AH, Gurney ME (2010) “Effects of 2,4-diaminoquinazoline derivatives on SMN expression and phenotype in a mouse model for spinal muscular atrophy” Hum Mol Genet.19:454-67.

• Burghes AH, Beattie CE, (2009) “Spinal muscular atrophy: why do low levels of survival motor neuron protein make motor neurons sick?” Nat Rev Neurosci. 10:597-609.

• Workman E, Saieva L, Carrel TL, Crawford TO, Liu D, Lutz C, Beattie CE, Pellizzoni L, Burghes AH (2009) “A SMN missense mutation complements SMN2 restoring snRNPs and rescuing SMA mice” Hum Mol Genet. 18:2215-29.

• McGovern VL, Gavrilina TO, Beattie CE and Burghes AH (2008) "Embryonic motor axon development in the severe SMA mouse" Hum Mol Genet 17(18):2900-9.

• Burghes AH and Butchbach ME (2008) "Let all DNA vote: who are "Neurology70(9):662-3.

• Thurmond J, Butchbach ME, Palomo M, Pease B, Rao M, Bedell L, Keyvan M, Pai G, Mishra R, Haraldsson M, Andresson T, Bragason G, Thosteinsdottir M, Bjornsson JM, Coovert DD, Burghes AH, Burney ME and Singh J (2008) "Synthesis and biological evaluation of novel 2,4-diaminoquinazoline derivatives as SMN2 promoter activators for the potential treatment of spinal muscular atrophy" J Med Chem 51(3):449-69.

• Gavrilina TO, McGovern VL, Workman E, Crawford TO, Gogliotto RG, DiDonato CJ, Monani UR, Morris GE and Burghes AH (2008) "Neuronal SMN expression corrects spinal muscular atrophy in severe SMA mice while muscle-specific SMN expression has no phenotypic effect" Hum Mol Genet. 17(8):1063-75.

• McWhorter ML, Boon KL, Horan ES, Burghes AH and Beattie CE (2008) "The SMN binding protein Gemin2 is not involved in motor axon outgrowth" Dev Neurobiol. 68(2):182-94.

• Novoyatleva T, Heinrich B, Tang Y, Benderska N, Butchbach ME, Lorson CL, Lorson MA, Ben-Dov C, Fehlbaum P, Bracco L, Burghes AH, Bollen M and Stamm S (2007) "Protein phosphatase 1 binds to the RNA recognition motif of several splicing factors and regulates alternative pre-mRNA processing" Hum Mol Genet 17(1):52-70.

• Gabanella F, Butchbach ME, Saieva L, Carissimi C, Burghes AH and Pellizzoni L (2007) "Ribonucleoprotein Assembly Defects Correlate with Spinal Muscular Atrophy Severity and Preferentially Affect a Subset of Spliceosomal snRNPs" PLoS ONE 2(9):e921.

• Hao le T, Fuller HR, Lam le T, Le TT, Burghes AH and Morris GE (2007) "Absence of gemin5 from SMN complexes in nuclear Cajal bodies" BMC Cell Biol 8:28.

• Butchbach ME, Edwards JD and Burghes AH (2007) "Abnormal motor phenotype in the SMNDelta7 mouse model of spinal muscular atrophy" Neurobiol Dis. 27(2):207-19.

• Butchbach ME, Edwards JD, Schussler KR and Burghes AH (2006) "A novel method for oral delivery of drug compounds to the neonatal SMNDelta7 mouse model of spinal muscular atrophy" J Neurosci Methods 161(2):285-90.

• Carrel TL, McWhorter ML, Workman E, Zhang H, Wolstencroft EC, Lorson C, Bassell GJ, Burghes AHM and Beattie CE (2006) "Survival Motor Neuron Function in motor axons is independent of functions required for small nuclear ribunocleoprotein biogenesis" Neuroscience 26(43):11014-22.

• Acharyya S, Butchbach ME, Sahenk Z, Wang H, Saji M, Carathers M, Ringel MD, Skipworth RJ, Fearon KC, Hollingsworth MA, Muscarella P, Burghes AH, Rafael-Fortney JA and Guttridge DC (2005) "Dystrophin glycoprotein complex dysfunction: a regulatory link between muscular dystrophy and cancer cachexia" Cancer Cell 8(5):421-32.

• Bertini E, Burghes A, Bushby K, Estournet-Mathiaud B, Finkel RS, Hughes RA, Iannaccone ST, Melki J, Mercuri E, Muntoni F, Voit T, Reitter B, Swoboda KJ, Tiziano D, Tizzano E, Topaloglu H, Wirth B and Zerres K (2005) 134th ENMC International Workshop: Outcome Measures and Treatment of Spinal Muscular Atrophy, Naarden, The Netherlands. Neuromuscul Disord 15:802-16.

• Sharma A, Lambrechts A, Hao le T, Le TT, Sewry CA, Ampe C, Burghes AH and Morris GE (2005) "A role for complexes of survival of motor neurons (SMN) protein with gemins and profilin in neurite-like cytoplasmic extensions of cultured nerve cells" Exp Cell Res 309(1):185-97.

• Jarecki J, Chen X, Bernardino A, Coovert DD, Whitney M, Burghes A, Stack J and Pollok BA (2005) "Diverse small-molecule modulators of SMN expression found by high-throughput compound screening: early leads towards a therapeutic for spinal muscular atrophy" Hum Mol Genet 14(14):2003-18.

• Le TT, Pham LT, Butchbach ME, Zhang HL, Monani UR, Coovert DD, Gavrilina TO, Xing L, Bassell GL and Burghes AHM (2005) "SMN?7, the major product of the centromeric survival motor neuron (SMN2) gene, extends survival in mice with spinal muscular atrophy and associates with full-length SMN" Human Mol Genet 14(6):845-57.

• Mimoun A, Le T, Ralph GS, Walmsley L, Monani UR, Lee DCP, Wilkes F, Mitrophanous KA, Kingsman SM, Burghes AHM and Mazarakis ND (2004) "Lentivector-mediated SMN replacement in a mouse model of spinal muscular atrophy" J Clin Invest 114:1726.