Skip Navigation

This Article
Right arrow Full Text Freely available
Right arrow FREE Full Text (PDF) Freely available
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in ISI Web of Science
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrow Search for citing articles in:
ISI Web of Science (143)
Right arrowRequest Permissions
Google Scholar
Right arrow Articles by Kaufmann, W. E.
Right arrow Articles by Moser, H. W.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Kaufmann, W. E.
Right arrow Articles by Moser, H. W.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

Cerebral Cortex, Vol. 10, No. 10, 981-991, October 2000
© 2000 Oxford University Press

Dendritic Anomalies in Disorders Associated with Mental Retardation

Walter E. Kaufmann1,2,3,4,5 and Hugo W. Moser2,3,5

1 Departments of Pathology, , 2 Neurology, , 3 Pediatrics, and , 4 Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine and the , 5 Kennedy Krieger Institute, 707 N. Broadway, Baltimore, MD 21205, USA

Dendritic abnormalities are the most consistent anatomical correlates of mental retardation (MR). Earliest descriptions included dendritic spine dysgenesis, which was first associated with unclassified MR, but can also be found in genetic syndromes associated with MR. Genetic disorders with well-defined dendritic anomalies involving branches and/or spines include Down, Rett and fragile-X syndromes. Cytoarchitectonic analyses also suggest dendritic pathology in Williams and Rubinstein-Taybi syndromes. Dendritic abnormalities appear to have syndrome-specific pathogenesis and evolution, which correlate to some extent with their cognitive profile. The significance of dendritic pathology in synaptic circuitry and the role of animal models in the study of MR-associated dendritic abnormalities are also discussed. Finally, a model of genotype to neurologic phenotype pathway in MR, centered in dendritic abnormalities, is postulated.


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?


This article has been cited by other articles:


Home page
 Arch NeurolHome page
F. F. Hamdan, J. Gauthier, S. Pellerin, S. Dobrzeniecka, C. Marineau, E. Fombonne, L. Mottron, R. G. Lafreniere, P. Drapeau, J. C. Lacaille, et al.
No Association Between SRGAP3/MEGAP Haploinsufficiency and Mental Retardation
Arch Neurol, May 1, 2009; 66(5): 675 - 676.
[Full Text] [PDF]

Home page
 Hum Mol GenetHome page
A.-M. Lepagnol-Bestel, A. Zvara, G. Maussion, F. Quignon, B. Ngimbous, N. Ramoz, S. Imbeaud, Y. Loe-Mie, K. Benihoud, N. Agier, et al.
DYRK1A interacts with the REST/NRSF-SWI/SNF chromatin remodelling complex to deregulate gene clusters involved in the neuronal phenotypic traits of Down syndrome
Hum. Mol. Genet., April 15, 2009; 18(8): 1405 - 1414.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
Y. Lu, F. Wang, Y. Li, J. Ferris, J.-A Lee, and F.-B. Gao
The Drosophila homologue of the Angelman syndrome ubiquitin ligase regulates the formation of terminal dendritic branches
Hum. Mol. Genet., February 1, 2009; 18(3): 454 - 462.
[Abstract] [Full Text] [PDF]

Home page
 J. Med. Genet.Home page
T V Bilousova, L Dansie, M Ngo, J Aye, J R Charles, D W Ethell, and I M Ethell
Minocycline promotes dendritic spine maturation and improves behavioural performance in the fragile X mouse model
J. Med. Genet., February 1, 2009; 46(2): 94 - 102.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
X.-M. Ma, D. D. Kiraly, E. D. Gaier, Y. Wang, E.-J. Kim, E. S. Levine, B. A. Eipper, and R. E. Mains
Kalirin-7 Is Required for Synaptic Structure and Function
J. Neurosci., November 19, 2008; 28(47): 12368 - 12382.
[Abstract] [Full Text] [PDF]

Home page
 Proc. Natl. Acad. Sci. USAHome page
N. Bastrikova, G. A. Gardner, J. M. Reece, A. Jeromin, and S. M. Dudek
Synapse elimination accompanies functional plasticity in hippocampal neurons
PNAS, February 26, 2008; 105(8): 3123 - 3127.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
A. Y. Hung, K. Futai, C. Sala, J. G. Valtschanoff, J. Ryu, M. A. Woodworth, F. L. Kidd, C. C. Sung, T. Miyakawa, M. F. Bear, et al.
Smaller Dendritic Spines, Weaker Synaptic Transmission, but Enhanced Spatial Learning in Mice Lacking Shank1
J. Neurosci., February 13, 2008; 28(7): 1697 - 1708.
[Abstract] [Full Text] [PDF]

Home page
 PediatricsHome page
N. Whittle, S. B. Sartori, M. Dierssen, G. Lubec, and N. Singewald
Fetal Down Syndrome Brains Exhibit Aberrant Levels of Neurotransmitters Critical for Normal Brain Development
Pediatrics, December 1, 2007; 120(6): e1465 - e1471.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
M. K. Sfakianos, A. Eisman, S. L. Gourley, W. D. Bradley, A. J. Scheetz, J. Settleman, J. R. Taylor, C. A. Greer, A. Williamson, and A. J. Koleske
Inhibition of Rho via Arg and p190RhoGAP in the Postnatal Mouse Hippocampus Regulates Dendritic Spine Maturation, Synapse and Dendrite Stability, and Behavior
J. Neurosci., October 10, 2007; 27(41): 10982 - 10992.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
A. Shalizi, P. M. Bilimoria, J. Stegmuller, B. Gaudilliere, Y. Yang, K. Shuai, and A. Bonni
PIASx Is a MEF2 SUMO E3 Ligase That Promotes Postsynaptic Dendritic Morphogenesis
J. Neurosci., September 12, 2007; 27(37): 10037 - 10046.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
Y. Furutani, H. Matsuno, M. Kawasaki, T. Sasaki, K. Mori, and Y. Yoshihara
Interaction between Telencephalin and ERM Family Proteins Mediates Dendritic Filopodia Formation
J. Neurosci., August 15, 2007; 27(33): 8866 - 8876.
[Abstract] [Full Text] [PDF]

Home page
 Proc. Natl. Acad. Sci. USAHome page
B. Ramos, B. Gaudilliere, A. Bonni, and G. Gill
Transcription factor Sp4 regulates dendritic patterning during cerebellar maturation
PNAS, June 5, 2007; 104(23): 9882 - 9887.
[Abstract] [Full Text] [PDF]

Home page
 Proc. Natl. Acad. Sci. USAHome page
K. F. Tolias, J. B. Bikoff, C. G. Kane, C. S. Tolias, L. Hu, and M. E. Greenberg
The Rac1 guanine nucleotide exchange factor Tiam1 mediates EphB receptor-dependent dendritic spine development
PNAS, April 24, 2007; 104(17): 7265 - 7270.
[Abstract] [Full Text] [PDF]

Home page
 Genes Dev.Home page
J. Z. Parrish, K. Emoto, L. Y. Jan, and Y. N. Jan
Polycomb genes interact with the tumor suppressor genes hippo and warts in the maintenance of Drosophila sensory neuron dendrites
Genes & Dev., April 15, 2007; 21(8): 956 - 972.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
B. E. Pfeiffer and K. M. Huber
Fragile X Mental Retardation Protein Induces Synapse Loss through Acute Postsynaptic Translational Regulation
J. Neurosci., March 21, 2007; 27(12): 3120 - 3130.
[Abstract] [Full Text] [PDF]

Home page
 Proc. Natl. Acad. Sci. USAHome page
G. Diana, G. Valentini, S. Travaglione, L. Falzano, M. Pieri, C. Zona, S. Meschini, A. Fabbri, and C. Fiorentini
Enhancement of learning and memory after activation of cerebral Rho GTPases
PNAS, January 9, 2007; 104(2): 636 - 641.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
I. Nishijima, T. Yamagata, C. M. Spencer, E. J. Weeber, O. Alekseyenko, J. D. Sweatt, M. Y. Momoi, M. Ito, D. L. Armstrong, D. L. Nelson, et al.
Secretin receptor-deficient mice exhibit impaired synaptic plasticity and social behavior
Hum. Mol. Genet., November 1, 2006; 15(21): 3241 - 3250.
[Abstract] [Full Text] [PDF]

Home page
 J Intellect DisabilHome page
V. Fodale, F. Mafrica, V. Caminiti, and G. Grasso
The cholinergic system in Down's syndrome.
J Intellect Disabil., September 1, 2006; 10(3): 261 - 274.
[Abstract] [PDF]

Home page
 J. Neurosci.Home page
M. Haber, L. Zhou, and K. K. Murai
Cooperative astrocyte and dendritic spine dynamics at hippocampal excitatory synapses.
J. Neurosci., August 30, 2006; 26(35): 8881 - 8891.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
R. Kraft, M. M. Escobar, M. L. Narro, J. L. Kurtis, A. Efrat, K. Barnard, and L. L. Restifo
Phenotypes of Drosophila Brain Neurons in Primary Culture Reveal a Role for Fascin in Neurite Shape and Trajectory.
J. Neurosci., August 23, 2006; 26(34): 8734 - 8747.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
H. Matsuno, S. Okabe, M. Mishina, T. Yanagida, K. Mori, and Y. Yoshihara
Telencephalin Slows Spine Maturation
J. Neurosci., February 8, 2006; 26(6): 1776 - 1786.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
E. M. Y. Moresco, S. Donaldson, A. Williamson, and A. J. Koleske
Integrin-Mediated Dendrite Branch Maintenance Requires Abelson (Abl) Family Kinases
J. Neurosci., June 29, 2005; 25(26): 6105 - 6118.
[Abstract] [Full Text] [PDF]

Home page
 Mol. Biol. CellHome page
R. T. Terry-Lorenzo, D. W. Roadcap, T. Otsuka, T. A. Blanpied, P. L. Zamorano, C. C. Garner, S. Shenolikar, and M. D. Ehlers
Neurabin/Protein Phosphatase-1 Complex Regulates Dendritic Spine Morphogenesis and Maturation
Mol. Biol. Cell, May 1, 2005; 16(5): 2349 - 2362.
[Abstract] [Full Text] [PDF]

Home page
 Cereb CortexHome page
T. L. Spires, Z. Molnar, P. C. Kind, P. M. Cordery, A. L. Upton, C. Blakemore, and A. J. Hannan
Activity-dependent Regulation of Synapse and Dendritic Spine Morphology in Developing Barrel Cortex Requires Phospholipase C-{beta}1 Signalling
Cereb Cortex, April 1, 2005; 15(4): 385 - 393.
[Abstract] [Full Text] [PDF]

Home page
 ScienceHome page
B. Chih, H. Engelman, and P. Scheiffele
Control of Excitatory and Inhibitory Synapse Formation by Neuroligins
Science, February 25, 2005; 307(5713): 1324 - 1328.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
B. Boda, S. Alberi, I. Nikonenko, R. Node-Langlois, P. Jourdain, M. Moosmayer, L. Parisi-Jourdain, and D. Muller
The Mental Retardation Protein PAK3 Contributes to Synapse Formation and Plasticity in Hippocampus
J. Neurosci., December 1, 2004; 24(48): 10816 - 10825.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
P. N. Lacor, M. C. Buniel, L. Chang, S. J. Fernandez, Y. Gong, K. L. Viola, M. P. Lambert, P. T. Velasco, E. H. Bigio, C. E. Finch, et al.
Synaptic Targeting by Alzheimer's-Related Amyloid {beta} Oligomers
J. Neurosci., November 10, 2004; 24(45): 10191 - 10200.
[Abstract] [Full Text] [PDF]

Home page
 NeuroscientistHome page
K. K. Murai and E. B. Pasquale
Eph Receptors, Ephrins, and Synaptic Function
Neuroscientist, August 1, 2004; 10(4): 304 - 314.
[Abstract] [PDF]

Home page
 GeneticsHome page
J. K. Inlow and L. L. Restifo
Molecular and Comparative Genetics of Mental Retardation
Genetics, February 1, 2004; 166(2): 835 - 881.
[Abstract] [Full Text] [PDF]

Home page
 JCBHome page
M. Henkemeyer, O. S. Itkis, M. Ngo, P. W. Hickmott, and I. M. Ethell
Multiple EphB receptor tyrosine kinases shape dendritic spines in the hippocampus
J. Cell Biol., December 22, 2003; 163(6): 1313 - 1326.
[Abstract] [Full Text] [PDF]

Home page
 J Child NeurolHome page
W. E. Kaufmann, K. L. Cooper, S. H. Mostofsky, G. T. Capone, W. R. Kates, C. J. Newschaffer, I. Bukelis, M. H. Stump, A. E. Jann, and D. C. Lanham
Specificity of Cerebellar Vermian Abnormalities in Autism: A Quantitative Magnetic Resonance Imaging Study
J Child Neurol, July 1, 2003; 18(7): 463 - 470.
[Abstract] [PDF]

Home page
 Cereb CortexHome page
M. Dierssen, R. Benavides-Piccione, C. Martinez-Cue, X. Estivill, J. Florez, G.N. Elston, and J. DeFelipe
Alterations of Neocortical Pyramidal Cell Phenotype in the Ts65Dn Mouse Model of Down Syndrome: Effects of Environmental Enrichment
Cereb Cortex, July 1, 2003; 13(7): 758 - 764.
[Abstract] [Full Text] [PDF]

Home page
 JCBHome page
H. Zhang, D. J. Webb, H. Asmussen, and A. F. Horwitz
Synapse formation is regulated by the signaling adaptor GIT1
J. Cell Biol., April 14, 2003; 161(1): 131 - 142.
[Abstract] [Full Text] [PDF]

Home page
 Cereb CortexHome page
W. E. Kaufmann, S. M. MacDonald, and C. R. Altamura
Dendritic Cytoskeletal Protein Expression in Mental Retardation: An Immunohistochemical Study of the Neocortex in Rett Syndrome
Cereb Cortex, October 1, 2000; 10(10): 992 - 1004.
[Abstract] [Full Text] [PDF]


Disclaimer: Please note that abstracts for content published before 1996 were created through digital scanning and may therefore not exactly replicate the text of the original print issues. All efforts have been made to ensure accuracy, but the Publisher will not be held responsible for any remaining inaccuracies. If you require any further clarification, please contact our Customer Services Department.