Differential involvement of striosome and matrix dopamine systems in a transgenic model of dopa-responsive dystonia.

Proceedings of the National Academy of Sciences of the United States of America
Kenta SatoSatoshi Goto

Abstract

Dopa-responsive dystonia (DRD) is a hereditary dystonia characterized by a childhood onset of fixed dystonic posture with a dramatic and sustained response to relatively low doses of levodopa. DRD is thought to result from striatal dopamine deficiency due to a reduced synthesis and activity of tyrosine hydroxylase (TH), the synthetic enzyme for dopamine. The mechanisms underlying the genesis of dystonia in DRD present a challenge to models of basal ganglia movement control, given that striatal dopamine deficiency is the hallmark of Parkinson's disease. We report here behavioral and anatomical observations on a transgenic mouse model for DRD in which the gene for 6-pyruvoyl-tetrahydropterin synthase is targeted to render selective dysfunction of TH synthesis in the striatum. Mutant mice exhibited motor deficits phenotypically resembling symptoms of human DRD and manifested a major depletion of TH labeling in the striatum, with a marked posterior-to-anterior gradient resulting in near total loss caudally. Strikingly, within the regions of remaining TH staining in the striatum, there was a greater loss of TH labeling in striosomes than in the surrounding matrix. The predominant loss of TH expression in striosomes occurred during t...Continue Reading

References

May 1, 1992·Proceedings of the National Academy of Sciences of the United States of America·R MoratallaA M Graybiel
Jul 1, 1990·Trends in Neurosciences·M R DeLong
Feb 1, 1995·Trends in Neurosciences·R L AlbinJ B Penney
Apr 1, 1994·Annals of Neurology·A H RajputO Hornykiewicz
May 1, 1997·Movement Disorders : Official Journal of the Movement Disorder Society·T HaniharaM Owada
Oct 1, 1998·Neurobiology of Learning and Memory·A M Graybiel
Mar 22, 2000·Nature Neuroscience·J J Canales, A M Graybiel
Oct 29, 2000·Trends in Neurosciences·A M GraybielC Capper-Loup
Oct 23, 2002·Neurology·D A GrimesD E Bulman
Jul 2, 2003·Annals of Neurology·Florence PasquierBruce L Miller
Jul 10, 2003·Lancet Neurology·Patricia M de Carvalho Aguiar, Laurie J Ozelius
Aug 2, 2003·Annals of Neurology·Masaya SegawaNobuyoshi Nishiyama
Aug 27, 2004·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Esen SakaAnn M Graybiel
May 25, 2005·Annals of Neurology·Satoshi GotoRyuji Kaji
Nov 8, 2005·Current Opinion in Neurobiology·Ann M Graybiel

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Citations

Feb 4, 2010·Journal of Neurology·Chul Hyoung Lyoo, Myung Sik Lee
Jan 28, 2009·Proceedings of the National Academy of Sciences of the United States of America·Jill R CrittendenAnn M Graybiel
Nov 11, 2010·The Journal of Biological Chemistry·Daigo HommaHiroshi Ichinose
Jul 7, 2009·Brain : a Journal of Neurology·Ulrich Müller
Apr 20, 2013·Brain : a Journal of Neurology·Satoshi GotoRyuji Kaji
Jul 16, 2010·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·F Asmus, T Gasser
Feb 10, 2009·Rinshō shinkeigaku = Clinical neurology·Ryuji KajiSatoshi Goto
Aug 4, 2012·Trends in Neurosciences·Aryn H Gittis, Anatol C Kreitzer
Feb 19, 2011·International Journal of Developmental Neuroscience : the Official Journal of the International Society for Developmental Neuroscience·Yoko TanimuraMark H Lewis
Dec 17, 2009·Neurobiology of Disease·David A PetersonHoward Poizner
Jun 15, 2015·Brain & Development·Kazue KimuraMasaya Segawa
Dec 10, 2015·Frontiers in Neuroanatomy·Ryoma Morigaki, Satoshi Goto
Jul 16, 2010·Journal of Neurochemistry·Li BaoMargaret E Rice
Sep 16, 2010·Movement Disorders : Official Journal of the Movement Disorder Society·Marek BodziochAndrzej Szczudlik
Jul 31, 2013·Movement Disorders : Official Journal of the Movement Disorder Society·Stéphane LehéricySabine Meunier
Apr 20, 2016·Frontiers in Neuroanatomy·Ryoma Morigaki, Satoshi Goto
Aug 27, 2011·The Biochemical Journal·Ernst R WernerBeat Thöny
Feb 24, 2015·Progress in Neurobiology·K L Eskow JaunarajsA Pisani
Jul 19, 2014·Progress in Neurobiology·Franziska Richter, Angelika Richter
Jun 8, 2017·Brain Sciences·Ryoma Morigaki, Satoshi Goto
Nov 2, 2017·Journal of Neurology, Neurosurgery, and Psychiatry·Ryuji KajiAnn M Graybiel
Jun 11, 2009·Current Opinion in Neurology·Marie VidailhetEmmanuel Roze
Aug 4, 2019·Annals of Neurology·Henrike HanssenNorbert Brüggemann
Apr 4, 2014·Internal Medicine·Natsumi FurutaKoichi Okamoto
Mar 13, 2018·Frontiers in Neurology·Angelo Quartarone, Diane Ruge
Oct 3, 2009·Annals of Neurology·Jeannette Vásquez-VivarSidhartha Tan
Jun 17, 2018·International Journal of Obesity : Journal of the International Association for the Study of Obesity·Máté DurstZsuzsanna E Tóth
Jan 25, 2021·Journal of Neural Transmission·Norbert Brüggemann

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