Corticostriatal network dysfunction in Huntington's disease: Deficits in neural processing, glutamate transport, and ascorbate release

CNS Neuroscience & Therapeutics
George V Rebec

Abstract

This review summarizes evidence for dysfunctional connectivity between cortical and striatal neurons in Huntington's disease (HD), a fatal neurodegenerative condition caused by a single gene mutation. The focus is on data derived from recording of electrophysiological signals in behaving transgenic mouse models. Firing patterns of individual neurons and the frequency oscillations of local field potentials indicate a disruption in corticostriatal processing driven, in large part, by interactions between cells that contain the mutant gene rather than the mutant gene alone. Dysregulation of glutamate, an excitatory amino acid released by cortical afferents, plays a key role in the breakdown of corticostriatal communication, a process modulated by ascorbate, an antioxidant vitamin found in high concentration in striatum. Up-regulation of glutamate transport by drug administration or viral-vector delivery improves ascorbate homeostasis and neurobehavioral processing in HD mice. Further analysis of electrophysiological data, including the use of sophisticated computational strategies, is required to discern how behavioral demands modulate the flow of corticostriatal information and its disruption by HD. Long before massive cell loss ...Continue Reading

References

Nov 1, 1985·Journal of Neuropathology and Experimental Neurology·J P VonsattelE P Richardson
Feb 12, 1982·Neuroscience Letters·K MilbyR N Adams
Jan 1, 1993·Neuroscience and Biobehavioral Reviews·J L HaraczG V Rebec
May 20, 1998·Journal of Neurophysiology·J R Wickens, C J Wilson
May 22, 1998·Journal of Neuropathology and Experimental Neurology·J P Vonsattel, M DiFiglia
May 23, 2001·Progress in Neurobiology·N C Danbolt
Oct 11, 2001·Neurobiology of Disease·J C LiévensG P Bates
Jan 1, 1991·The European Journal of Neuroscience·Tyra ZetterströmMarianne Fillenz
Jul 24, 2002·Brain : a Journal of Neurology·P F BehrensG B Landwehrmeyer
Feb 20, 2003·Trends in Neurosciences·Eugene M IzhikevichFrank C Hoppensteadt
Oct 4, 2003·Cytogenetic and Genome Research·M A Hickey, M-F Chesselet
Mar 12, 2004·Progress in Neurobiology·Izhar Bar-GadHagai Bergman
Nov 24, 2004·Neuroscience and Biobehavioral Reviews·Jeffrey W DalleyTrevor W Robbins
May 23, 2006·Biological Psychiatry·Gregory J QuirkFrancisco González-Lima
Aug 30, 2007·Neurochemical Research·Bjørnar HasselPiers C Emson
Oct 18, 2007·Proceedings of the National Academy of Sciences of the United States of America·M DiFigliaN Aronin
Oct 30, 2007·Nature Neuroscience·Christian S Lobsiger, Don W Cleveland
Dec 22, 2007·Journal of Neurology, Neurosurgery, and Psychiatry·J S PaulsenUNKNOWN Predict-HD Investigators and Coordinators of the Huntington Study Group
Jun 14, 2008·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Michelle GrayX William Yang
Jun 19, 2008·Annual Review of Neuroscience·Ann M Graybiel
Jul 1, 2008·Experimental Neurology·Robert Christian WolfBernhard Landwehrmeyer
Sep 5, 2008·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Adam G WalkerGeorge V Rebec
Dec 10, 2008·Neuroscience·C BeurrierP Gubellini
Dec 17, 2008·Proceedings of the National Academy of Sciences of the United States of America·Adriano B L TortNancy J Kopell
Dec 23, 2008·Nature Biotechnology·Kevin D FoustBrian K Kaspar
Feb 10, 2009·Biological Psychiatry·Sven SchipplingMichael Orth
Mar 5, 2009·Disease Models & Mechanisms·Dagmar E EhrnhoeferMichael R Hayden

❮ Previous
Next ❯

Citations

Mar 28, 2018·CNS Neuroscience & Therapeutics·Carlos Cepeda, Xiao-Ping Tong
Nov 24, 2019·Journal of Neuroscience Research·Wei Li, Lucas Pozzo-Miller
Sep 19, 2019·Neurotherapeutics : the Journal of the American Society for Experimental NeuroTherapeutics·Jordi Creus-Muncunill, Michelle E Ehrlich
Apr 21, 2020·Journal of Huntington's Disease·Caodu BurenLynn A Raymond
Aug 17, 2018·International Journal of Molecular Sciences·Abraham Rosas-ArellanoMaite A Castro
Mar 3, 2020·Frontiers in Neuroscience·Sonja Blumenstock, Irina Dudanova
Mar 2, 2021·Frontiers in Cellular Neuroscience·Guendalina BergonzoniMarta Biagioli
Aug 3, 2021·Neurotherapeutics : the Journal of the American Society for Experimental NeuroTherapeutics·María E PradosEduardo Muñoz

❮ Previous
Next ❯

Related Concepts

Related Feeds

Cell-Type-Specific Viral Vectors (ASM)

Viral vectors are used in biological research and therapy to deliver genetic material into cells. However, the efficiency of viral vectors varies depending on the cell type. Here is the latest research on cell-type-specific viral vectors.

Cell-Type-Specific Viral Vectors

Viral vectors are used in biological research and therapy to deliver genetic material into cells. However, the efficiency of viral vectors varies depending on the cell type. Here is the latest research on cell-type-specific viral vectors.

Cell-Type Specific Viral Vectors

Viral vectors are used in biological research and therapy to deliver genetic material into cells. However, the efficiency of viral vectors varies depending on the cell type. Here is the latest research on cell-type-specific viral vectors.

Basal Ganglia

Basal Ganglia are a group of subcortical nuclei in the brain associated with control of voluntary motor movements, procedural and habit learning, emotion, and cognition. Here is the latest research.