The transcriptional repressor Mecp2 regulates terminal neuronal differentiation

Molecular and Cellular Neurosciences
Valéry MatarazzoGabriele V Ronnett

Abstract

Rett syndrome (RTT) is a severe neurodevelopmental disorder with features of autism that results from mutation of the gene encoding the transcriptional repressor methyl-CpG binding protein (MECP2). The consequences of loss of a transcription factor may be complex, affecting the expression of many proteins, thus limiting understanding of this class of diseases and impeding therapeutic strategies. This is true for RTT. Neither the cell biological mechanism(s) nor the developmental stage affected by MECP2 deficiency is known. In vivo analysis of the olfactory system demonstrates that Mecp2 deficiency leads to a transient delay in the terminal differentiation of olfactory neurons. This delay in maturation disrupts axonal targeting in the olfactory bulb, resulting in abnormal axonal projections, subglomerular disorganization, and a persistent reduction in glomerular size. These results indicate a critical cell biological function for Mecp2 in mediating the final stages of neuronal development.

References

Sep 1, 1976·The Journal of Comparative Neurology·J W Hinds, P L Hinds
Sep 1, 1976·The Journal of Comparative Neurology·J W Hinds, P L Hinds
Oct 10, 1975·Brain Research·B K Hartman, F L Margolis
Jun 1, 1992·Journal of Autism and Developmental Disorders·G Woodyatt, A Ozanne
Jan 1, 1991·Progress in Brain Research·F L MargolisM Grillo
Jan 1, 1990·Brain & Development·Y Nomura, M Segawa
Jan 1, 1987·Brain & Development·A M KerrJ B Stephenson
Mar 1, 1987·Proceedings of the National Academy of Sciences of the United States of America·K E RogersF L Margolis
Oct 1, 1972·Physiological Reviews·G M Shepherd
Apr 20, 1983·The Journal of Comparative Neurology·R M Costanzo, P P Graziadei
Apr 1, 1982·Archives of Otolaryngology·M A LovellJ C Rowley
May 1, 1995·Developmental Dynamics : an Official Publication of the American Association of Anatomists·J M Huard, J E Schwob
Mar 1, 1995·Journal of Neuropathology and Experimental Neurology·D ArmstrongR Trivedi
Dec 1, 1993·Experimental Neurology·V M Carr, A I Farbman
Jun 1, 1993·American Journal of Diseases of Children·R J SchultzA K Percy
Aug 26, 1998·Journal of Neurobiology·A L CalofJ Shou
Nov 24, 1998·Journal of Neuropathology and Experimental Neurology·D D ArmstrongB Antalffy
Mar 19, 1999·Pediatric Neurology·D D ArmstrongK J Motil
Apr 23, 1999·The Journal of Comparative Neurology·H J KasowskiC A Greer
May 14, 1999·Current Opinion in Genetics & Development·H H Ng, A Bird
Oct 19, 1999·The Journal of Comparative Neurology·H B TreloarC A Greer
Jun 8, 2000·The Journal of Comparative Neurology·H Kim, C A Greer
Jun 28, 2000·Progress in Neurobiology·A Mackay-Sima, M I Chuahb
Oct 11, 2001·Neurobiology of Disease·S AkbarianE G Jones
Dec 12, 2001·Brain & Development·E C Azmitia
Dec 12, 2001·Brain & Development·H G Dunn
Dec 12, 2001·Brain & Development·M V JohnstonS Naidu
Jun 27, 2002·Proceedings of the National Academy of Sciences of the United States of America·Cheil MoonGabriele V Ronnett
Jan 1, 1990·The European Journal of Neuroscience·J. VerhaagenF. L. Margolis

❮ Previous
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Citations

Oct 24, 2009·Behavior Genetics·Jean-Christophe Roux, Laurent Villard
May 19, 2006·Cellular and Molecular Neurobiology·Danette J NicolayAdil J Nazarali
Apr 29, 2005·Cell Research·Guoping Fan, Leah Hutnick
May 25, 2006·European Journal of Human Genetics : EJHG·Jamel ChellyThierry Bienvenu
Mar 6, 2010·Human Molecular Genetics·Matías Alvarez-SaavedraJuan I Young
Jan 18, 2011·Experimental Biology and Medicine·Gaston CalfaLucas Pozzo-Miller
Mar 1, 2012·PloS One·Fernando VonhoffCarsten Duch
May 16, 2013·European Journal of Human Genetics : EJHG·Frederic Tran Mau-ThemDavid Genevieve
Mar 13, 2014·Neuromolecular Medicine·Vichithra R B Liyanage, Mojgan Rastegar
Jul 5, 2011·Annual Review of Cell and Developmental Biology·Jacky GuyAdrian Bird
Dec 24, 2011·Pediatrics and Neonatology·Shih-Ming WengStuart R Cobb
Jan 7, 2016·Frontiers in Cellular Neuroscience·Wan Y LeongEyleen L K Goh
May 10, 2011·Neurobiology of Learning and Memory·Tamara B Franklin, Isabelle M Mansuy
Mar 2, 2010·Molecular and Cellular Neurosciences·Jessica L MacdonaldA Jane Roskams
Apr 13, 2011·Journal of Neurodevelopmental Disorders·Ege T KavalaliLisa M Monteggia
Jun 26, 2009·Progress in Neurobiology·Jessica L MacDonald, A Jane Roskams
Feb 17, 2009·Neurobiology of Disease·Jennifer L LarimoreLucas Pozzo-Miller
Dec 9, 2008·Biological Psychiatry·Lisa M Monteggia, Ege T Kavalali
Sep 9, 2011·The Anatomical Record : Advances in Integrative Anatomy and Evolutionary Biology·Mary E BlueMichael V Johnston
Apr 1, 2011·The Journal of Comparative Neurology·Matthew J EcklerBin Chen
May 2, 2006·Current Opinion in Genetics & Development·Paolo Moretti, Huda Y Zoghbi
Jul 25, 2009·Molecular and Cellular Neurosciences·Alicia L DeganoGabriele V Ronnett
Jan 30, 2014·Molecular and Cellular Neurosciences·Alicia L DeganoGabriele V Ronnett
Nov 9, 2007·Neuron·Maria Chahrour, Huda Y Zoghbi
Oct 31, 2015·Cell Regeneration·Heather McGowan, Zhiping P Pang
Oct 18, 2005·Journal of Child Neurology·Dawna Duncan Armstrong
Oct 18, 2005·Journal of Child Neurology·Noriyuki Kishi, Jeffrey D Macklis
Oct 18, 2005·Journal of Child Neurology·Michael V JohnstonSakkubai Naidu
Aug 26, 2006·Nature Clinical Practice. Neurology·Uta Francke
Apr 6, 2005·Human Molecular Genetics·Isabel Martín Caballero, Brian Hendrich
Jun 17, 2005·Biochemistry and Cell Biology = Biochimie Et Biologie Cellulaire·Jordanka Zlatanova
Nov 26, 2016·Orphanet Journal of Rare Diseases·Friederike EhrhartLeopold M G Curfs
Mar 14, 2018·Molecular Neurobiology·Godwin SokporTran Tuoc
Jul 25, 2019·Frontiers in Cellular Neuroscience·Maria Laura BertoldiAlicia L Degano
Mar 26, 2005·Current Opinion in Neurology·Masaya Segawa, Yoshiko Nomura
Apr 15, 2006·Human Molecular Genetics·Jinglan Liu, Uta Francke
Jun 28, 2005·Journal of Neuropathology and Experimental Neurology·Tetsuya FukudaYu-ichi Goto

❮ Previous
Next ❯

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