Publications

  1. Huang, YT, Crick, HR, Chaytow, H, van der Hoorn, D, Alhindi, A, Jones, RA et al.. Long-term muscle-specific overexpression of DOK7 in mice using AAV9-tMCK-DOK7. Mol Ther Nucleic Acids. 2023;33 :617-628. doi: 10.1016/j.omtn.2023.07.036. PubMed PMID:37637210 PubMed Central PMC10457688.
  2. Powers, S, Likhite, S, Gadalla, KK, Miranda, CJ, Huffenberger, AJ, Dennys, C et al.. Novel MECP2 gene therapy is effective in a multicenter study using two mouse models of Rett syndrome and is safe in non-human primates. Mol Ther. 2023;31 (9):2767-2782. doi: 10.1016/j.ymthe.2023.07.013. PubMed PMID:37481701 PubMed Central PMC10492029.
  3. Zlatic, SA, Duong, D, Gadalla, KKE, Murage, B, Ping, L, Shah, R et al.. Convergent cerebrospinal fluid proteomes and metabolic ontologies in humans and animal models of Rett syndrome. iScience. 2022;25 (9):104966. doi: 10.1016/j.isci.2022.104966. PubMed PMID:36060065 PubMed Central PMC9437849.
  4. Kim, JY, Bai, Y, Jayne, LA, Hector, RD, Persaud, AK, Ong, SS et al.. A kinome-wide screen identifies a CDKL5-SOX9 regulatory axis in epithelial cell death and kidney injury. Nat Commun. 2020;11 (1):1924. doi: 10.1038/s41467-020-15638-6. PubMed PMID:32317630 PubMed Central PMC7174303.
  5. Bernardo, P, Cobb, S, Coppola, A, Tomasevic, L, Di Lazzaro, V, Bravaccio, C et al.. Neurophysiological Signatures of Motor Impairment in Patients with Rett Syndrome. Ann Neurol. 2020;87 (5):763-773. doi: 10.1002/ana.25712. PubMed PMID:32129908 .
  6. Cobb, SR. Disrupted inhibition may give clues in understanding neurodevelopmental disorders. Eur J Paediatr Neurol. 2020;24 :3. doi: 10.1016/j.ejpn.2020.01.012. PubMed PMID:31973984 .
  7. Velasco-Estevez, M, Gadalla, KKE, Liñan-Barba, N, Cobb, S, Dev, KK, Sheridan, GK et al.. Inhibition of Piezo1 attenuates demyelination in the central nervous system. Glia. 2020;68 (2):356-375. doi: 10.1002/glia.23722. PubMed PMID:31596529 .
  8. Turko, P, Groberman, K, Browa, F, Cobb, S, Vida, I. Differential Dependence of GABAergic and Glutamatergic Neurons on Glia for the Establishment of Synaptic Transmission. Cereb Cortex. 2019;29 (3):1230-1243. doi: 10.1093/cercor/bhy029. PubMed PMID:29425353 .
  9. Hector, RD, Kalscheuer, VM, Hennig, F, Leonard, H, Downs, J, Clarke, A et al.. CDKL5 variants: Improving our understanding of a rare neurologic disorder. Neurol Genet. 2017;3 (6):e200. doi: 10.1212/NXG.0000000000000200. PubMed PMID:29264392 PubMed Central PMC5732004.
  10. Tillotson, R, Selfridge, J, Koerner, MV, Gadalla, KKE, Guy, J, De Sousa, D et al.. Radically truncated MeCP2 rescues Rett syndrome-like neurological defects. Nature. 2017;550 (7676):398-401. doi: 10.1038/nature24058. PubMed PMID:29019980 PubMed Central PMC5884422.
  11. Bahey, NG, Gadalla, KKE, McGonigal, R, Bailey, MES, Edgar, JM, Cobb, SR et al.. Reduced axonal diameter of peripheral nerve fibers in a mouse model of Rett syndrome. Neuroscience. 2017;358 :261-268. doi: 10.1016/j.neuroscience.2017.06.061. PubMed PMID:28687309 .
  12. Gadalla, KKE, Vudhironarit, T, Hector, RD, Sinnett, S, Bahey, NG, Bailey, MES et al.. Development of a Novel AAV Gene Therapy Cassette with Improved Safety Features and Efficacy in a Mouse Model of Rett Syndrome. Mol Ther Methods Clin Dev. 2017;5 :180-190. doi: 10.1016/j.omtm.2017.04.007. PubMed PMID:28497075 PubMed Central PMC5423329.
  13. Sinnett, SE, Hector, RD, Gadalla, KKE, Heindel, C, Chen, D, Zaric, V et al.. Improved MECP2 Gene Therapy Extends the Survival of MeCP2-Null Mice without Apparent Toxicity after Intracisternal Delivery. Mol Ther Methods Clin Dev. 2017;5 :106-115. doi: 10.1016/j.omtm.2017.04.006. PubMed PMID:28497072 PubMed Central PMC5424572.
  14. Ross, PD, Guy, J, Selfridge, J, Kamal, B, Bahey, N, Tanner, KE et al.. Exclusive expression of MeCP2 in the nervous system distinguishes between brain and peripheral Rett syndrome-like phenotypes. Hum Mol Genet. 2016;25 (20):4389-4404. doi: 10.1093/hmg/ddw269. PubMed PMID:28173151 PubMed Central PMC5886038.
  15. Hector, RD, Dando, O, Ritakari, TE, Kind, PC, Bailey, ME, Cobb, SR et al.. Characterisation of Cdkl5 transcript isoforms in rat. Gene. 2017;603 :21-26. doi: 10.1016/j.gene.2016.12.001. PubMed PMID:27940108 .
  16. Leonard, H, Cobb, S, Downs, J. Clinical and biological progress over 50 years in Rett syndrome. Nat Rev Neurol. 2017;13 (1):37-51. doi: 10.1038/nrneurol.2016.186. PubMed PMID:27934853 .
  17. Forrest, CM, Kennedy, PG, Rodgers, J, Dalton, RN, Turner, C, Darlington, LG et al.. Kynurenine pathway metabolism following prenatal KMO inhibition and in Mecp2+/- mice, using liquid chromatography-tandem mass spectrometry. Neurochem Int. 2016;100 :110-119. doi: 10.1016/j.neuint.2016.09.012. PubMed PMID:27623092 PubMed Central PMC5115650.
  18. Hector, RD, Dando, O, Landsberger, N, Kilstrup-Nielsen, C, Kind, PC, Bailey, ME et al.. Characterisation of CDKL5 Transcript Isoforms in Human and Mouse. PLoS One. 2016;11 (6):e0157758. doi: 10.1371/journal.pone.0157758. PubMed PMID:27315173 PubMed Central PMC4912119.
  19. Cobb, SR. Cognitive disorders: Deep brain stimulation for Rett syndrome. Nature. 2015;526 (7573):331-2. doi: 10.1038/526331a. PubMed PMID:26469040 .
  20. Trägårdh, J, Robb, G, Gadalla, KK, Cobb, S, Travis, C, Oppo, GL et al.. Label-free imaging of thick tissue at 1550 nm using a femtosecond optical parametric generator. Opt Lett. 2015;40 (15):3484-7. doi: 10.1364/OL.40.003484. PubMed PMID:26258338 .
  21. Booker, SA, Pires, N, Cobb, S, Soares-da-Silva, P, Vida, I. Carbamazepine and oxcarbazepine, but not eslicarbazepine, enhance excitatory synaptic transmission onto hippocampal CA1 pyramidal cells through an antagonist action at adenosine A1 receptors. Neuropharmacology. 2015;93 :103-15. doi: 10.1016/j.neuropharm.2015.01.019. PubMed PMID:25656478 .
  22. Kamal, B, Russell, D, Payne, A, Constante, D, Tanner, KE, Isaksson, H et al.. Biomechanical properties of bone in a mouse model of Rett syndrome. Bone. 2015;71 :106-14. doi: 10.1016/j.bone.2014.10.008. PubMed PMID:25445449 PubMed Central PMC4289916.
  23. Gadalla, KK, Ross, PD, Riddell, JS, Bailey, ME, Cobb, SR. Gait analysis in a Mecp2 knockout mouse model of Rett syndrome reveals early-onset and progressive motor deficits. PLoS One. 2014;9 (11):e112889. doi: 10.1371/journal.pone.0112889. PubMed PMID:25392929 PubMed Central PMC4231076.
  24. Saudargiene, A, Cobb, S, Graham, BP. A computational study on plasticity during theta cycles at Schaffer collateral synapses on CA1 pyramidal cells in the hippocampus. Hippocampus. 2015;25 (2):208-18. doi: 10.1002/hipo.22365. PubMed PMID:25220633 .
  25. Graham, BP, Saudargiene, A, Cobb, S. Spine head calcium as a measure of summed postsynaptic activity for driving synaptic plasticity. Neural Comput. 2014;26 (10):2194-222. doi: 10.1162/NECO_a_00640. PubMed PMID:25058697 .
  26. Robinson, L, Plano, A, Cobb, S, Riedel, G. Long-term home cage activity scans reveal lowered exploratory behaviour in symptomatic female Rett mice. Behav Brain Res. 2013;250 :148-56. doi: 10.1016/j.bbr.2013.04.041. PubMed PMID:23643691 PubMed Central PMC3885800.
  27. Cobb, SR, Davies, CH. Neurodevelopmental disorders. Neuropharmacology. 2013;68 :1. doi: 10.1016/j.neuropharm.2013.02.001. PubMed PMID:23402709 .
  28. Gadalla, KK, Bailey, ME, Spike, RC, Ross, PD, Woodard, KT, Kalburgi, SN et al.. Improved survival and reduced phenotypic severity following AAV9/MECP2 gene transfer to neonatal and juvenile male Mecp2 knockout mice. Mol Ther. 2013;21 (1):18-30. doi: 10.1038/mt.2012.200. PubMed PMID:23011033 PubMed Central PMC3536818.
  29. Robinson, L, Guy, J, McKay, L, Brockett, E, Spike, RC, Selfridge, J et al.. Morphological and functional reversal of phenotypes in a mouse model of Rett syndrome. Brain. 2012;135 (Pt 9):2699-710. doi: 10.1093/brain/aws096. PubMed PMID:22525157 PubMed Central PMC3437019.
  30. Weng, SM, Bailey, ME, Cobb, SR. Rett syndrome: from bed to bench. Pediatr Neonatol. 2011;52 (6):309-16. doi: 10.1016/j.pedneo.2011.08.002. PubMed PMID:22192257 .
  31. Barberan, S, McNair, K, Iqbal, K, Smith, NC, Prendergast, GC, Stone, TW et al.. Altered apoptotic responses in neurons lacking RhoB GTPase. Eur J Neurosci. 2011;34 (11):1737-46. doi: 10.1111/j.1460-9568.2011.07891.x. PubMed PMID:22098422 PubMed Central PMC4384704.
  32. Gadalla, KK, Bailey, ME, Cobb, SR. MeCP2 and Rett syndrome: reversibility and potential avenues for therapy. Biochem J. 2011;439 (1):1-14. doi: 10.1042/BJ20110648. PubMed PMID:21916843 .
  33. Nathan, PJ, Cobb, SR, Lu, B, Bullmore, ET, Davies, CH. Studying synaptic plasticity in the human brain and opportunities for drug discovery. Curr Opin Pharmacol. 2011;11 (5):540-8. doi: 10.1016/j.coph.2011.06.008. PubMed PMID:21737346 .
  34. Poland, SP, Wright, AJ, Cobb, S, Vijverberg, JC, Girkin, JM. A demonstration of the effectiveness of a single aberration correction per optical slice in beam scanned optically sectioning microscopes. Micron. 2011;42 (4):318-23. doi: 10.1016/j.micron.2010.05.018. PubMed PMID:20932768 .
  35. Cobb, S, Guy, J, Bird, A. Reversibility of functional deficits in experimental models of Rett syndrome. Biochem Soc Trans. 2010;38 (2):498-506. doi: 10.1042/BST0380498. PubMed PMID:20298210 .
  36. McNair, K, Spike, R, Guilding, C, Prendergast, GC, Stone, TW, Cobb, SR et al.. A role for RhoB in synaptic plasticity and the regulation of neuronal morphology. J Neurosci. 2010;30 (9):3508-17. doi: 10.1523/JNEUROSCI.5386-09.2010. PubMed PMID:20203211 PubMed Central PMC6634083.
  37. Cutsuridis, V, Cobb, S, Graham, BP. Encoding and retrieval in a model of the hippocampal CA1 microcircuit. Hippocampus. 2010;20 (3):423-46. doi: 10.1002/hipo.20661. PubMed PMID:19489002 .
  38. Goodwin, RJ, Dungworth, JC, Cobb, SR, Pitt, AR. Time-dependent evolution of tissue markers by MALDI-MS imaging. Proteomics. 2008;8 (18):3801-8. doi: 10.1002/pmic.200800201. PubMed PMID:18712770 .
  39. Cobb, SR, Pitt, A. Proteomics in the study of hippocampal plasticity. Expert Rev Proteomics. 2008;5 (3):393-404. doi: 10.1586/14789450.5.3.393. PubMed PMID:18532907 .
  40. Yau, JL, McNair, KM, Noble, J, Brownstein, D, Hibberd, C, Morton, N et al.. Enhanced hippocampal long-term potentiation and spatial learning in aged 11beta-hydroxysteroid dehydrogenase type 1 knock-out mice. J Neurosci. 2007;27 (39):10487-96. doi: 10.1523/JNEUROSCI.2190-07.2007. PubMed PMID:17898220 PubMed Central PMC6673151.
  41. Shelbourne, P, Coote, E, Dadak, S, Cobb, SR. Normal electrical properties of hippocampal neurons modelling early Huntington disease pathogenesis. Brain Res. 2007;1139 :226-34. doi: 10.1016/j.brainres.2006.12.091. PubMed PMID:17291464 .
  42. Guy, J, Gan, J, Selfridge, J, Cobb, S, Bird, A. Reversal of neurological defects in a mouse model of Rett syndrome. Science. 2007;315 (5815):1143-7. doi: 10.1126/science.1138389. PubMed PMID:17289941 PubMed Central PMC7610836.
  43. Bushell, TJ, Plevin, R, Cobb, S, Irving, AJ. Characterization of proteinase-activated receptor 2 signalling and expression in rat hippocampal neurons and astrocytes. Neuropharmacology. 2006;50 (6):714-25. doi: 10.1016/j.neuropharm.2005.11.024. PubMed PMID:16430928 .
  44. Gill, CH, Brown, JT, Shivji, N, Lappin, SC, Farmer, C, Randall, A et al.. Inhibition of Ih reduces epileptiform activity in rodent hippocampal slices. Synapse. 2006;59 (5):308-16. doi: 10.1002/syn.20242. PubMed PMID:16421904 .
  45. McNair, K, Davies, CH, Cobb, SR. Plasticity-related regulation of the hippocampal proteome. Eur J Neurosci. 2006;23 (2):575-80. doi: 10.1111/j.1460-9568.2005.04542.x. PubMed PMID:16420465 .
  46. Cobb, SR, Davies, CH. Cholinergic modulation of hippocampal cells and circuits. J Physiol. 2005;562 (Pt 1):81-8. doi: 10.1113/jphysiol.2004.076539. PubMed PMID:15528238 PubMed Central PMC1665493.
  47. Lanneau, C, Harries, MH, Ray, AM, Cobb, SR, Randall, A, Davies, CH et al.. Complex interactions between mGluR1 and mGluR5 shape neuronal network activity in the rat hippocampus. Neuropharmacology. 2002;43 (2):131-40. doi: 10.1016/s0028-3908(02)00086-2. PubMed PMID:12213267 .
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