About this Journal Submit a Manuscript Table of Contents
Journal of Biomedicine and Biotechnology
Volume 2010 (2010), Article ID 849426, 19 pages
http://dx.doi.org/10.1155/2010/849426
Review Article

Dystrophins, Utrophins, and Associated Scaffolding Complexes: Role in Mammalian Brain and Implications for Therapeutic Strategies

1Univ Paris-Sud, Centre de Neurosciences Paris-Sud, UMR8195, Orsay, 91405, France
2CNRS, Orsay, 91405, France

Received 16 December 2009; Accepted 14 March 2010

Academic Editor: Meena Upadhyaya

Copyright © 2010 Caroline Perronnet and Cyrille Vaillend. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Citations to this Article [28 citations]

The following is the list of published articles that have cited the current article.

  • Cyrille Vaillend, Caroline Perronnet, Carine Ros, Carole Gruszczynski, Aurélie Goyenvalle, Serge Laroche, Olivier Danos, Luis Garcia, and Elise Peltekian, “Rescue of a dystrophin-like protein by exon skipping in vivo restores GABAA-receptor clustering in the hippocampus of the mdx mouse.,” Molecular therapy : the journal of the American Society of Gene Therapy, vol. 18, no. 9, pp. 1683–8, 2010. View at Publisher · View at Google Scholar
  • Caroline Perronnet, and Cyrille Vaillend, “Dystrophins, Utrophins, and Associated Scaffolding Complexes: Role in Mammalian Brain and Implications for Therapeutic Strategies (849426, 2010),” Journal Of Biomedicine And Biotechnology, 2010. View at Publisher · View at Google Scholar
  • April K. Marrone, Mariya M. Kucherenko, Valentyna M. Rishko, and Halyna R. Shcherbata, “New Dystrophin/Dystroglycan interactors control neuron behavior in Drosophila eye,” Bmc Neuroscience, vol. 12, 2011. View at Publisher · View at Google Scholar
  • Kueh, Dempster, Head, and Morley, “Reduced postsynaptic GABAA receptor number and enhanced gaboxadol induced change in holding currents in Purkinje cells of the dystrophin-deficient mdx mouse,” Neurobiology of Disease, vol. 43, no. 3, pp. 558–564, 2011. View at Publisher · View at Google Scholar
  • Glenn Dallerac, Caroline Perronnet, Carine Chagneau, Pascale Leblanc-Veyrac, Nathalie Samson-Desvignes, Elise Peltekian, Olivier Danos, Luis Garcia, Serge Laroche, Jean-Marie Billard, and Cyrille Vaillend, “Rescue of a dystrophin-like protein by exon skipping normalizes synaptic plasticity in the hippocampus of the mdx mouse,” Neurobiology Of Disease, vol. 43, no. 3, pp. 635–641, 2011. View at Publisher · View at Google Scholar
  • Ruben Miranda, Uri Nudel, Serge Laroche, and Cyrille Vaillend, “Altered presynaptic ultrastructure in excitatory hippocampal synapses of mice lacking dystrophins Dp427 or Dp71,” Neurobiology Of Disease, vol. 43, no. 1, pp. 134–141, 2011. View at Publisher · View at Google Scholar
  • Marco Sassoe-Pognetto, Elena Frola, Giulia Pregno, Federica Briatore, and Annarita Patrizi, “Understanding the molecular diversity of GABAergic synapses,” Frontiers in Cellular Neuroscience, vol. 5, 2011. View at Publisher · View at Google Scholar
  • Qi Bing, Jing Hu, Na Li, Zhe Zhao, Hong-Rui Shen, Jun-Hui Yuan, and Yan Liu, “Clinical and glucocorticosteroid therapeutic analyses of 96 patients with Duchenne muscular dystrophy,” Chinese Journal of Neurology, vol. 44, no. 11, pp. 745–749, 2011. View at Publisher · View at Google Scholar
  • Patrizia Panzanelli, Benjamin G. Gunn, Monika C. Schlatter, Dietmar Benke, Shiva K. Tyagarajan, Peter Scheiffele, Delia Belelli, Jeremy J. Lambert, Uwe Rudolph, and Jean-Marc Fritschy, “Distinct mechanisms regulate GABA(A) receptor and gephyrin clustering at perisomatic and axo-axonic synapses on CA1 pyramidal cells,” Journal Of Physiology-London, vol. 589, no. 20, pp. 4959–4980, 2011. View at Publisher · View at Google Scholar
  • Richard A. Zuellig, Beat C. Bornhauser, Ralf Amstutz, Bruno Constantin, and Marcus C. Schaub, “Tissue Expression and Actin Binding of a Novel N-Terminal Utrophin Isoform,” Journal of Biomedicine and Biotechnology, vol. 2011, pp. 1–18, 2011. View at Publisher · View at Google Scholar
  • Adrian Waite, Susan C. Brown, and Derek J. Blake, “The dystrophin–glycoprotein complex in brain development and disease,” Trends in Neurosciences, vol. 35, no. 8, pp. 487–496, 2012. View at Publisher · View at Google Scholar
  • Patrizia Panzanelli, Shiva K. Tyagarajan, and Jean-Marc Fritschy, “Molecular and functional heterogeneity of GABAergic synapses,” Cellular and Molecular Life Sciences, vol. 69, no. 15, pp. 2485–2499, 2012. View at Publisher · View at Google Scholar
  • Caroline Perronnet, Carine Chagneau, Pascale Le Blanc, Nathalie Samson-Desvignes, Dominique Mornet, Serge Laroche, Sabine De La Porte, and Cyrille Vaillend, “Upregulation of brain utrophin does not rescue behavioral alterations in dystrophin-deficient mice,” Human Molecular Genetics, vol. 21, no. 10, pp. 2263–2276, 2012. View at Publisher · View at Google Scholar
  • Maaike van Putten, Darshan Kumar, Margriet Hulsker, Willem M. H. Hoogaars, Jaap J. Plomp, Annemarieke van Opstal, Maarten van Iterson, Peter Admiraal, Gert-Jan B. van Ommen, Peter A. C. 't Hoen, and Annemieke Aartsma-Rus, “Comparison of skeletal muscle pathology and motor function of dystrophin and utrophin deficient mouse strains,” Neuromuscular Disorders, vol. 22, no. 5, pp. 406–417, 2012. View at Publisher · View at Google Scholar
  • Milena Cau, Loredana Boccone, Anna Mateddu, Maria Addis, Marianna Serrenti, Roberta Chessa, Gianni Marrosu, Georgios Loudianos, and Maria Antonietta Melis, “A new deletion in 5 '-end of dystrophin gene removing M and P promoters and dystrophin muscle enhancers,” Gene, vol. 511, no. 2, pp. 437–440, 2012. View at Publisher · View at Google Scholar
  • Paulo Cesar Ghedini, Maria Christina W. Avellar, Thereza Christina M. De Lima, Maria Teresa R. Lima-Landman, Antonio Jose Lapa, and Caden Souccar, “Quantitative changes of nicotinic receptors in the hippocampus of dystrophin-deficient mice,” Brain Research, vol. 1483, pp. 96–104, 2012. View at Publisher · View at Google Scholar
  • Xiaoli Zhao, Joseph G. Moloughney, Sai Zhang, Shinji Komazaki, and Noah Weisleder, “Orai1 Mediates Exacerbated Ca2+ Entry in Dystrophic Skeletal Muscle,” Plos One, vol. 7, no. 11, 2012. View at Publisher · View at Google Scholar
  • Laure Fresard, Sophie Leroux, Patrice Dehais, Bertrand Servin, Helene Gilbert, Olivier Bouchez, Christophe Klopp, Cedric Cabau, Florence Vignoles, Katia Feve, Amelie Ricros, David Gourichon, Christian Diot, Sabine Richard, Christine Leterrier, Catherine Beaumont, Alain Vignal, Francis Minvielle, and Frederique Pitel, “Fine mapping of complex traits in non-model species: using next generation sequencing and advanced intercross lines in Japanese quail,” Bmc Genomics, vol. 13, 2012. View at Publisher · View at Google Scholar
  • Wanda M. Snow, Judy E. Anderson, and Lorna S. Jakobson, “Neuropsychological and neurobehavioral functioning in Duchenne muscular dystrophy: A review,” Neuroscience and Biobehavioral Reviews, vol. 37, no. 5, pp. 743–752, 2013. View at Publisher · View at Google Scholar
  • Wanda M. Snow, Mark Fry, and Judy E. Anderson, “Increased density of dystrophin protein in the lateral versus the vermal mouse cerebellum,” Cellular and Molecular Neurobiology, vol. 33, no. 4, pp. 513–520, 2013. View at Publisher · View at Google Scholar
  • Richard A. Jensen, Barbara E. K. Klein, Lenore J. Launer, Albert Vernon Smith, Eric Boerwinkle, Ning Cheung, Alex W. Hewitt, Gerald Liew, Paul Mitchell, Jie Jin Wang, John Attia, Xueling Sim, Rodney Scott, Nicole L. Glazer, Thomas Lumley, Barbara McKnight, Bruce M. Psaty, Kent Taylor, Albert Hofman, Paulus T. V. M. de Jong, Fernando Rivadeneira, Andre G. Uitterlinden, Xiaohui Li, Wan-Ting Tay, Yik Ying Teo, Mark Seielstad, Jianjun Liu, Ching-Yu Cheng, Seang-Mei Saw, Tin Aung, Santhi K. Ganesh, Christopher J. O'Donnell, Mike A. Nalls, Mary Frances Cotch, Kerri L. Wiggins, Jane Z. Kuo, Cornelia M. van Duijn, Vilmundur Gudnason, Ronald Klein, David S. Siscovick, Jerome I. Rotter, E. Shong Tai, Johannes Vingerling, Tien Y. Wong, M. Kamran Ikram, Elizabeth G. Holliday, Gudny Eiriksdottir, Tamara B. Harris, and Fridbert Jonasson, “Genome-Wide Association Study of Retinopathy in Individuals without Diabetes,” PLoS ONE, vol. 8, no. 2, 2013. View at Publisher · View at Google Scholar
  • Alyssa Janke, Ritika Upadhaya, Wanda M. Snow, and Judy E. Anderson, “A New Look at Cytoskeletal NOS-1 and beta-Dystroglycan Changes in Developing Muscle and Brain in Control and mdx Dystrophic Mice,” Developmental Dynamics, vol. 242, no. 12, pp. 1369–1381, 2013. View at Publisher · View at Google Scholar
  • Javier Costas, Jose Javier Suarez-Rama, Noa Carrera, Eduardo Paz, Mario Paramo, Santiago Agra, Julio Brenlla, Ramon Ramos-Rios, and Manuel Arrojo, “Role of DISC1 Interacting Proteins in Schizophrenia Risk from Genome-Wide Analysis of Missense SNPs,” Annals of Human Genetics, vol. 77, pp. 504–512, 2013. View at Publisher · View at Google Scholar
  • Nhi Tan, and Jeffry B. Lansman, “Utrophin regulates modal gating of mechanosensitive ion channels in dystrophic skeletal muscle,” Journal of Physiology-London, vol. 592, no. 15, pp. 3303–3323, 2014. View at Publisher · View at Google Scholar
  • S. F. Parames, E. D. Coletta-Yudice, F. M. Nogueira, M. B. Nering De Sousa, M. A. Hayashi, M. T. R. Lima-Landman, and A. J. Lapa, “Altered Acetylcholine Release In The Hippocampus Of Dystrophin-Deficient Mice,” Neuroscience, vol. 269, pp. 173–183, 2014. View at Publisher · View at Google Scholar
  • Arjan P. M. de Brouwer, Sander B. Nabuurs, Ingrid E. C. Verhaart, Astrid R. Oudakker, Roel Hordijk, Helger G. Yntema, Jannet M. Hordijk-Hos, Krysta Voesenek, Bert B. A. de Vries, Ton van Essen, Wei Chen, Hao Hu, Jamel Chelly, Johan T. den Dunnen, Vera M. Kalscheuer, Annemieke M. Aartsma-Rus, Ben C. J. Hamel, Hans van Bokhoven, and Tjitske Kleefstra, “A 3-base pair deletion, c.9711_9713del, in DMD results in intellectual disability without muscular dystrophy,” European Journal of Human Genetics, vol. 22, no. 4, pp. 480–485, 2014. View at Publisher · View at Google Scholar
  • Wanda M. Snow, Judy E. Anderson, and Mark Fry, “Regional and genotypic differences in intrinsic electrophysiological properties of cerebellar Purkinje neurons from wild-type and dystrophin-deficient mdx mice,” Neurobiology of Learning and Memory, vol. 107, pp. 19–31, 2014. View at Publisher · View at Google Scholar
  • Antoni Parcerisas, Sara E. Rubio, Ashraf Muhaisen, Alberto Gomez-Ramos, Lluis Pujadas, Montserrat Puiggros, Daniela Rossi, Jesus Urena, Ferran Burgaya, Marta Pascual, David Torrents, Alberto Rabano, Jesus Avila, and Eduardo Soriano, “Somatic Signature of Brain-Specific Single Nucleotide Variations in Sporadic Alzheimer's Disease,” Journal of Alzheimers Disease, vol. 42, no. 4, pp. 1357–1382, 2014. View at Publisher · View at Google Scholar