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HSP90 Inhibitor, NVP-AUY922, Improves Myelination in Vitro and Supports the Maintenance of Myelinated Axons in Neuropathic Mice.

Chittoor-Vinod VG, Bazick H, Todd AG, Falk D, Morelli KH, Burgess RW, Foster TC, Notterpek L.

ACS Chem Neurosci. 2019 Jun 19;10(6):2890-2902. doi: 10.1021/acschemneuro.9b00105. Epub 2019 May 3.


Model validity for preclinical studies in precision medicine: precisely how precise do we need to be?

Tadenev ALD, Burgess RW.

Mamm Genome. 2019 Jun;30(5-6):111-122. doi: 10.1007/s00335-019-09798-0. Epub 2019 Apr 5.


Editorial: Axonopathy in Neurodegenerative Disease.

Burgess RW, Crish SD.

Front Neurosci. 2018 Oct 23;12:769. doi: 10.3389/fnins.2018.00769. eCollection 2018. No abstract available.


DSCAM promotes self-avoidance in the developing mouse retina by masking the functions of cadherin superfamily members.

Garrett AM, Khalil A, Walton DO, Burgess RW.

Proc Natl Acad Sci U S A. 2018 Oct 23;115(43):E10216-E10224. doi: 10.1073/pnas.1809430115. Epub 2018 Oct 8.


Aberrant GlyRS-HDAC6 interaction linked to axonal transport deficits in Charcot-Marie-Tooth neuropathy.

Mo Z, Zhao X, Liu H, Hu Q, Chen XQ, Pham J, Wei N, Liu Z, Zhou J, Burgess RW, Pfaff SL, Caskey CT, Wu C, Bai G, Yang XL.

Nat Commun. 2018 Mar 8;9(1):1007. doi: 10.1038/s41467-018-03461-z.


Accumulating Evidence for Axonal Translation in Neuronal Homeostasis.

Spaulding EL, Burgess RW.

Front Neurosci. 2017 May 31;11:312. doi: 10.3389/fnins.2017.00312. eCollection 2017. Review.


Severity of Demyelinating and Axonal Neuropathy Mouse Models Is Modified by Genes Affecting Structure and Function of Peripheral Nodes.

Morelli KH, Seburn KL, Schroeder DG, Spaulding EL, Dionne LA, Cox GA, Burgess RW.

Cell Rep. 2017 Mar 28;18(13):3178-3191. doi: 10.1016/j.celrep.2017.03.009.


Trk receptor signaling and sensory neuron fate are perturbed in human neuropathy caused by Gars mutations.

Sleigh JN, Dawes JM, West SJ, Wei N, Spaulding EL, Gómez-Martín A, Zhang Q, Burgess RW, Cader MZ, Talbot K, Yang XL, Bennett DL, Schiavo G.

Proc Natl Acad Sci U S A. 2017 Apr 18;114(16):E3324-E3333. doi: 10.1073/pnas.1614557114. Epub 2017 Mar 28.


Replacing the PDZ-interacting C-termini of DSCAM and DSCAML1 with epitope tags causes different phenotypic severity in different cell populations.

Garrett AM, Tadenev AL, Hammond YT, Fuerst PG, Burgess RW.

Elife. 2016 Sep 16;5. pii: e16144. doi: 10.7554/eLife.16144.


Metabolite profile of a mouse model of Charcot-Marie-Tooth type 2D neuropathy: implications for disease mechanisms and interventions.

Bais P, Beebe K, Morelli KH, Currie ME, Norberg SN, Evsikov AV, Miers KE, Seburn KL, Guergueltcheva V, Kremensky I, Jordanova A, Bult CJ, Burgess RW.

Biol Open. 2016 Jul 15;5(7):908-20. doi: 10.1242/bio.019273.


Neuromuscular Disease Models and Analysis.

Burgess RW, Cox GA, Seburn KL.

Methods Mol Biol. 2016;1438:349-94. doi: 10.1007/978-1-4939-3661-8_19.


Synaptic Deficits at Neuromuscular Junctions in Two Mouse Models of Charcot-Marie-Tooth Type 2d.

Spaulding EL, Sleigh JN, Morelli KH, Pinter MJ, Burgess RW, Seburn KL.

J Neurosci. 2016 Mar 16;36(11):3254-67. doi: 10.1523/JNEUROSCI.1762-15.2016.


Analysis of Expression Pattern and Genetic Deletion of Netrin5 in the Developing Mouse.

Garrett AM, Jucius TJ, Sigaud LP, Tang FL, Xiong WC, Ackerman SL, Burgess RW.

Front Mol Neurosci. 2016 Jan 26;9:3. doi: 10.3389/fnmol.2016.00003. eCollection 2016.


Corrigendum: Impaired protein translation in Drosophila models for Charcot-Marie-Tooth neuropathy caused by mutant tRNA synthetases.

Niehues S, Bussmann J, Steffes G, Erdmann I, Köhrer C, Sun L, Wagner M, Schäfer K, Wang G, Koerdt SN, Stum M, Jaiswal S, RajBhandary UL, Thomas U, Aberle H, Burgess RW, Yang XL, Dieterich D, Storkebaum E.

Nat Commun. 2016 Jan 21;7:10497. doi: 10.1038/ncomms10497. No abstract available.


Corrigendum: CMT2D neuropathy is linked to the neomorphic binding activity of glycyl-tRNA synthetase.

He W, Bai G, Zhou H, Wei N, White NM, Lauer J, Liu H, Shi Y, Dan Dumitru C, Lettieri K, Shubayev V, Jordanova A, Guergueltcheva V, Griffin PR, Burgess RW, Pfaff SL, Yang XL.

Nature. 2016 Apr 21;532(7599):402. doi: 10.1038/nature16499. Epub 2016 Jan 20. No abstract available.


Novel axon projection after stress and degeneration in the Dscam mutant retina.

Fernandes KA, Bloomsburg SJ, Miller CJ, Billingslea SA, Merrill MM, Burgess RW, Libby RT, Fuerst PG.

Mol Cell Neurosci. 2016 Mar;71:1-12. doi: 10.1016/j.mcn.2015.12.003. Epub 2015 Dec 10.


CMT2D neuropathy is linked to the neomorphic binding activity of glycyl-tRNA synthetase.

He W, Bai G, Zhou H, Wei N, White NM, Lauer J, Liu H, Shi Y, Dumitru CD, Lettieri K, Shubayev V, Jordanova A, Guergueltcheva V, Griffin PR, Burgess RW, Pfaff SL, Yang XL.

Nature. 2015 Oct 29;526(7575):710-4. doi: 10.1038/nature15510. Epub 2015 Oct 21. Erratum in: Nature. 2016 Apr 21;532(7599):402.


Self-awareness in the retina.

Garrett AM, Burgess RW.

Elife. 2015 Aug 25;4. doi: 10.7554/eLife.10233.


Impaired protein translation in Drosophila models for Charcot-Marie-Tooth neuropathy caused by mutant tRNA synthetases.

Niehues S, Bussmann J, Steffes G, Erdmann I, Köhrer C, Sun L, Wagner M, Schäfer K, Wang G, Koerdt SN, Stum M, Jaiswal S, RajBhandary UL, Thomas U, Aberle H, Burgess RW, Yang XL, Dieterich D, Storkebaum E.

Nat Commun. 2015 Jul 3;6:7520. doi: 10.1038/ncomms8520. Erratum in: Nat Commun. 2016;7:10497. Jaiswal, Sumit [Added].


Dominant, toxic gain-of-function mutations in gars lead to non-cell autonomous neuropathology.

Grice SJ, Sleigh JN, Motley WW, Liu JL, Burgess RW, Talbot K, Cader MZ.

Hum Mol Genet. 2015 Aug 1;24(15):4397-406. doi: 10.1093/hmg/ddv176. Epub 2015 May 13.


A brief review of recent Charcot-Marie-Tooth research and priorities.

Ekins S, Litterman NK, Arnold RJ, Burgess RW, Freundlich JS, Gray SJ, Higgins JJ, Langley B, Willis DE, Notterpek L, Pleasure D, Sereda MW, Moore A.

F1000Res. 2015 Feb 26;4:53. doi: 10.12688/f1000research.6160.1. eCollection 2015. Review.


The MuSK activator agrin has a separate role essential for postnatal maintenance of neuromuscular synapses.

Tezuka T, Inoue A, Hoshi T, Weatherbee SD, Burgess RW, Ueta R, Yamanashi Y.

Proc Natl Acad Sci U S A. 2014 Nov 18;111(46):16556-61. doi: 10.1073/pnas.1408409111. Epub 2014 Nov 3.


Lack of neuropathy-related phenotypes in hint1 knockout mice.

Seburn KL, Morelli KH, Jordanova A, Burgess RW.

J Neuropathol Exp Neurol. 2014 Jul;73(7):693-701. doi: 10.1097/NEN.0000000000000085.


Morphological analysis of neuromuscular junction development and degeneration in rodent lumbrical muscles.

Sleigh JN, Burgess RW, Gillingwater TH, Cader MZ.

J Neurosci Methods. 2014 Apr 30;227:159-65. doi: 10.1016/j.jneumeth.2014.02.005. Epub 2014 Feb 14.


Neuromuscular junction maturation defects precede impaired lower motor neuron connectivity in Charcot-Marie-Tooth type 2D mice.

Sleigh JN, Grice SJ, Burgess RW, Talbot K, Cader MZ.

Hum Mol Genet. 2014 May 15;23(10):2639-50. doi: 10.1093/hmg/ddt659. Epub 2013 Dec 23.


Introduction to mechanisms of neural circuit formation.

Weiner JA, Jontes JD, Burgess RW.

Front Mol Neurosci. 2013 May 13;6:12. doi: 10.3389/fnmol.2013.00012. eCollection 2013. No abstract available.


Loss of the E3 ubiquitin ligase LRSAM1 sensitizes peripheral axons to degeneration in a mouse model of Charcot-Marie-Tooth disease.

Bogdanik LP, Sleigh JN, Tian C, Samuels ME, Bedard K, Seburn KL, Burgess RW.

Dis Model Mech. 2013 May;6(3):780-92. doi: 10.1242/dmm.010942. Epub 2013 Mar 8.


A novel mouse Dscam mutation inhibits localization and shedding of DSCAM.

Schramm RD, Li S, Harris BS, Rounds RP, Burgess RW, Ytreberg FM, Fuerst PG.

PLoS One. 2012;7(12):e52652. doi: 10.1371/journal.pone.0052652. Epub 2012 Dec 26.


DSCAMs: restoring balance to developmental forces.

Garrett AM, Tadenev AL, Burgess RW.

Front Mol Neurosci. 2012 Aug 17;5:86. doi: 10.3389/fnmol.2012.00086. eCollection 2012.


Neuronal clustering and fasciculation phenotype in Dscam- and Bax-deficient mouse retinas.

Keeley PW, Sliff BJ, Lee SC, Fuerst PG, Burgess RW, Eglen SJ, Reese BE.

J Comp Neurol. 2012 May 1;520(7):1349-64. doi: 10.1002/cne.23033.


A MusD retrotransposon insertion in the mouse Slc6a5 gene causes alterations in neuromuscular junction maturation and behavioral phenotypes.

Bogdanik LP, Chapman HD, Miers KE, Serreze DV, Burgess RW.

PLoS One. 2012;7(1):e30217. doi: 10.1371/journal.pone.0030217. Epub 2012 Jan 17.


Contact is repulsive, but please note the "enclosed".

Burgess RW, Garrett AM, Tadenev AL.

Dev Cell. 2012 Jan 17;22(1):5-6. doi: 10.1016/j.devcel.2011.12.017.


A spontaneous mutation in contactin 1 in the mouse.

Davisson MT, Bronson RT, Tadenev AL, Motley WW, Krishnaswamy A, Seburn KL, Burgess RW.

PLoS One. 2011;6(12):e29538. doi: 10.1371/journal.pone.0029538. Epub 2011 Dec 29.


Charcot-Marie-Tooth-linked mutant GARS is toxic to peripheral neurons independent of wild-type GARS levels.

Motley WW, Seburn KL, Nawaz MH, Miers KE, Cheng J, Antonellis A, Green ED, Talbot K, Yang XL, Fischbeck KH, Burgess RW.

PLoS Genet. 2011 Dec;7(12):e1002399. doi: 10.1371/journal.pgen.1002399. Epub 2011 Dec 1.


Changes in brain β-amyloid deposition and aquaporin 4 levels in response to altered agrin expression in mice.

Rauch SM, Huen K, Miller MC, Chaudry H, Lau M, Sanes JR, Johanson CE, Stopa EG, Burgess RW.

J Neuropathol Exp Neurol. 2011 Dec;70(12):1124-37. doi: 10.1097/NEN.0b013e31823b0b12.


Cell autonomy of DSCAM function in retinal development.

Fuerst PG, Bruce F, Rounds RP, Erskine L, Burgess RW.

Dev Biol. 2012 Jan 15;361(2):326-37. doi: 10.1016/j.ydbio.2011.10.028. Epub 2011 Oct 29.


A novel mouse model of Niemann-Pick type C disease carrying a D1005G-Npc1 mutation comparable to commonly observed human mutations.

Maue RA, Burgess RW, Wang B, Wooley CM, Seburn KL, Vanier MT, Rogers MA, Chang CC, Chang TY, Harris BT, Graber DJ, Penatti CA, Porter DM, Szwergold BS, Henderson LP, Totenhagen JW, Trouard TP, Borbon IA, Erickson RP.

Hum Mol Genet. 2012 Feb 15;21(4):730-50. doi: 10.1093/hmg/ddr505. Epub 2011 Nov 2.


A valid mouse model of AGRIN-associated congenital myasthenic syndrome.

Bogdanik LP, Burgess RW.

Hum Mol Genet. 2011 Dec 1;20(23):4617-33. doi: 10.1093/hmg/ddr396. Epub 2011 Sep 1.


Candidate molecular mechanisms for establishing cell identity in the developing retina.

Garrett AM, Burgess RW.

Dev Neurobiol. 2011 Dec;71(12):1258-72. doi: 10.1002/dneu.20926. Review.


The Down syndrome critical region regulates retinogeniculate refinement.

Blank M, Fuerst PG, Stevens B, Nouri N, Kirkby L, Warrier D, Barres BA, Feller MB, Huberman AD, Burgess RW, Garner CC.

J Neurosci. 2011 Apr 13;31(15):5764-76. doi: 10.1523/JNEUROSCI.6015-10.2011.


A mouse model of heritable cerebrovascular disease.

Sproule TJ, Sled JG, Wentzell J, Wang B, Henkelman RM, Roopenian DC, Burgess RW.

PLoS One. 2010 Dec 31;5(12):e15327. doi: 10.1371/journal.pone.0015327.


An assessment of mechanisms underlying peripheral axonal degeneration caused by aminoacyl-tRNA synthetase mutations.

Stum M, McLaughlin HM, Kleinbrink EL, Miers KE, Ackerman SL, Seburn KL, Antonellis A, Burgess RW.

Mol Cell Neurosci. 2011 Feb;46(2):432-43. doi: 10.1016/j.mcn.2010.11.006. Epub 2010 Nov 27.


Two-photon NADH imaging exposes boundaries of oxygen diffusion in cortical vascular supply regions.

Kasischke KA, Lambert EM, Panepento B, Sun A, Gelbard HA, Burgess RW, Foster TH, Nedergaard M.

J Cereb Blood Flow Metab. 2011 Jan;31(1):68-81. doi: 10.1038/jcbfm.2010.158. Epub 2010 Sep 22.


A novel null allele of mouse DSCAM survives to adulthood on an inbred C3H background with reduced phenotypic variability.

Fuerst PG, Harris BS, Johnson KR, Burgess RW.

Genesis. 2010 Oct 1;48(10):578-84. doi: 10.1002/dvg.20662.


Neuromuscular disease models and analysis.

Burgess RW, Cox GA, Seburn KL.

Methods Mol Biol. 2010;602:347-93. doi: 10.1007/978-1-60761-058-8_20.


Rab3 dynamically controls protein composition at active zones.

Graf ER, Daniels RW, Burgess RW, Schwarz TL, DiAntonio A.

Neuron. 2009 Dec 10;64(5):663-77. doi: 10.1016/j.neuron.2009.11.002.


DSCAM and DSCAML1 function in self-avoidance in multiple cell types in the developing mouse retina.

Fuerst PG, Bruce F, Tian M, Wei W, Elstrott J, Feller MB, Erskine L, Singer JH, Burgess RW.

Neuron. 2009 Nov 25;64(4):484-97. doi: 10.1016/j.neuron.2009.09.027.


Adhesion molecules in establishing retinal circuitry.

Fuerst PG, Burgess RW.

Curr Opin Neurobiol. 2009 Aug;19(4):389-94. doi: 10.1016/j.conb.2009.07.013. Epub 2009 Aug 5. Review.


An ENU-induced mutation in mouse glycyl-tRNA synthetase (GARS) causes peripheral sensory and motor phenotypes creating a model of Charcot-Marie-Tooth type 2D peripheral neuropathy.

Achilli F, Bros-Facer V, Williams HP, Banks GT, AlQatari M, Chia R, Tucci V, Groves M, Nickols CD, Seburn KL, Kendall R, Cader MZ, Talbot K, van Minnen J, Burgess RW, Brandner S, Martin JE, Koltzenburg M, Greensmith L, Nolan PM, Fisher EM.

Dis Model Mech. 2009 Jul-Aug;2(7-8):359-73. doi: 10.1242/dmm.002527. Epub 2009 May 26.

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