On the cause of mental retardation in Down syndrome: extrapolation from full and segmental trisomy 16 mouse models

Brain Research. Brain Research Reviews
Zygmunt GaldzickiS I Rapoport

Abstract

Down syndrome (DS, trisomy 21, Ts21) is the most common known cause of mental retardation. In vivo structural brain imaging in young DS adults, and post-mortem studies, indicate a normal brain size after correction for height, and the absence of neuropathology. Functional imaging with positron emission tomography (PET) shows normal brain glucose metabolism, but fewer significant correlations between metabolic rates in different brain regions than in controls, suggesting reduced functional connections between brain circuit elements. Cultured neurons from Ts21 fetuses and from fetuses of an animal model for DS, the trisomy 16 (Ts16) mouse, do not differ from controls with regard to passive electrical membrane properties, including resting potential and membrane resistance. On the other hand, the trisomic neurons demonstrate abnormal active electrical and biochemical properties (duration of action potential and its rates of depolarization and repolarization, altered kinetics of active Na(+), Ca(2+) and K(+) currents, altered membrane densities of Na(+) and Ca(2+) channels). Another animal model, the adult segmental trisomy 16 mouse (Ts65Dn), demonstrates reduced long-term potentiation and increased long-term depression (models for...Continue Reading

References

Jan 1, 1978·Journal of Neuropathology and Experimental Neurology·R S WilliamsV S Caviness
May 1, 1976·The Journal of Comparative Neurology·M Marin-Padilla
Sep 1, 1977·Proceedings of the National Academy of Sciences of the United States of America·D Schubert, C Whitlock
Jan 1, 1975·Cytogenetics and Cell Genetics·A GroppD Giers
Sep 1, 1992·Trends in Neurosciences·S O Meakin, E M Shooter
Nov 1, 1992·The Journal of Pediatrics·P D Walson
Jan 1, 1991·Experimental Brain Research·W BryschK H Schlingensiepen
Feb 1, 1990·Developmental Biology·J D RobackB H Wainer
May 1, 1990·Trends in Neurosciences·M Stewart, S E Fox
Jun 4, 1990·Brain Research·D M FinchM B Jackson
Nov 11, 1991·Neuroscience Letters·C A ColtonM L Oster-Granite
Feb 15, 1992·Proceedings of the National Academy of Sciences of the United States of America·D M HoltzmanW C Mobley
Mar 1, 1991·Archives of Neurology·S M PueschelP McKnight
Mar 1, 1991·Developmental Medicine and Child Neurology·C E StafstromK E Wisniewski
Nov 1, 1991·Pediatric Pathology·T MitoL E Becker
Mar 1, 1991·Proceedings of the National Academy of Sciences of the United States of America·S FisherM L Oster-Granite
Jan 1, 1991·The International Journal of Neuroscience·Z GaldzickiM Sciancalepore
Apr 1, 1990·Brain Research. Molecular Brain Research·M T CasertaJ T Coyle
Jan 1, 1990·Advances in Experimental Medicine and Biology·S Fisher, M L Oster-Granite
Mar 1, 1991·Proceedings of the National Academy of Sciences of the United States of America·P Corsi, J T Coyle
Oct 1, 1990·Journal of Neuroscience Methods·P CaviedesS I Rapoport
Jun 11, 1990·Brain Research·C A ColtonM L Oster-Granite
Jan 1, 1990·Annual Review of Cell Biology·R W Tsien, R Y Tsien
Sep 1, 1986·Proceedings of the National Academy of Sciences of the United States of America·T M PerneyR J Miller
Nov 1, 1986·Biochemistry and Cell Biology = Biochimie Et Biologie Cellulaire·J K Reed, D England
Mar 1, 1988·The Journal of General Physiology·P SahP W Gage

❮ Previous
Next ❯

Citations

Jun 24, 2004·Neurobiology of Aging·Christopher L HunterAnn-Charlotte Granholm
May 7, 2003·Neuroscience and Biobehavioral Reviews·Igor BranchiLaura Ricceri
Jun 28, 2002·Brain Research. Brain Research Reviews·John C FialaKristen M Harris
Mar 19, 2003·Behavioural Brain Research·Heather A Bimonte-NelsonAnn-Charlotte E Granholm
Jan 16, 2003·Behavioural Brain Research·Christopher L HunterAnn Charlotte E Granholm
Sep 6, 2003·Free Radical Biology & Medicine·Toby N Behar, Carol A Colton
Dec 19, 2009·Cell Death and Differentiation·L BaroncelliL Maffei
Aug 2, 2006·Genes, Brain, and Behavior·C Martínez-CuéJ Flórez
May 1, 2012·Current Gerontology and Geriatrics Research·Jason P LockrowAnn-Charlotte E Granholm
Aug 15, 2012·Cellular and Molecular Life Sciences : CMLS·Qiang ZhangDahai Zhu
Sep 29, 2011·Journal of Chemical Neuroanatomy·Stefano GottiGianCarlo Panzica
Dec 2, 2008·Experimental and Molecular Pathology·Seymour LevineStephen D Ginsberg
Aug 23, 2003·Genes, Brain, and Behavior·Z Galdzicki, R J Siarey
Feb 27, 2003·Journal of Neurochemistry·Ephrem Engidawork, Gert Lubec
Oct 22, 2005·Mental Retardation and Developmental Disabilities Research Reviews·Tarik F Haydar
Nov 27, 2008·The Journal of Comparative Neurology·Pavel V BelichenkoWilliam C Mobley
Mar 11, 2011·Journal of Neurochemistry·Robert A Rissman, William C Mobley
May 2, 2006·Journal of Physiology, Paris·Katherine SaudPablo Caviedes
Apr 16, 2013·Clinical Neurophysiology : Official Journal of the International Federation of Clinical Neurophysiology·Mehran AhmadlouFiroozeh Sajedi
Aug 1, 2015·PloS One·Pavel V BelichenkoWilliam C Mobley
Jun 28, 2017·American Journal on Intellectual and Developmental Disabilities·Pasqualina M PicciottiGraziano Onder
Nov 10, 2006·Journal of Neurophysiology·Tyler K BestZygmunt Galdzicki
Jul 9, 2009·Physiological Reviews·Mara DierssenXavier Estivill
Aug 23, 2003·Genes, Brain, and Behavior·A Bhattacharyya, C N Svendsen
Mar 24, 2007·Molecular Biology Reports·Wang XinWu Qihan

❮ Previous
Next ❯

Related Concepts

Related Feeds

Brain Imaging of Neural Circuits

Neural circuits are groups of interconnected neurons which carry out specific functions when activated. Imaging these neural circuits allows researches to further elucidate their mechanisms and functions. Follow this feed to stay up to date on brain imaging of neural circuits.