R6/2 Huntington's disease mice develop early and progressive abnormal brain metabolism and seizures.

The Journal of Neuroscience : the Official Journal of the Society for Neuroscience
Efrain Cepeda-PradoHerman Moreno

Abstract

A hallmark feature of Huntington's disease pathology is the atrophy of brain regions including, but not limited to, the striatum. Though MRI studies have identified structural CNS changes in several Huntington's disease (HD) mouse models, the functional consequences of HD pathology during the progression of the disease have yet to be investigated using in vivo functional MRI (fMRI). To address this issue, we first established the structural and functional MRI phenotype of juvenile HD mouse model R6/2 at early and advanced stages of disease. Significantly higher fMRI signals [relative cerebral blood volumes (rCBVs)] and atrophy were observed in both age groups in specific brain regions. Next, fMRI results were correlated with electrophysiological analysis, which showed abnormal increases in neuronal activity in affected brain regions, thus identifying a mechanism accounting for the abnormal fMRI findings. [(14)C] 2-deoxyglucose maps to investigate patterns of glucose utilization were also generated. An interesting mismatch between increases in rCBV and decreases in glucose uptake was observed. Finally, we evaluated the sensitivity of this mouse line to audiogenic seizures early in the disease course. We found that R6/2 mice had ...Continue Reading

References

Nov 1, 1985·Journal of Neuropathology and Experimental Neurology·J P VonsattelE P Richardson
Mar 1, 1972·Electroencephalography and Clinical Neurophysiology·R J Racine
Nov 17, 1983·Nature·J F GusellaA Y Sakaguchi
Jun 1, 1994·Magnetic Resonance in Medicine : Official Journal of the Society of Magnetic Resonance in Medicine·R M WeisskoffB R Rosen
Jan 27, 1998·Neurology·E H AylwardC A Ross
May 22, 1998·Journal of Neuropathology and Experimental Neurology·J P Vonsattel, M DiFiglia
Oct 6, 1999·Lancet·D C CostaP J Ell
May 17, 2000·Proceedings of the National Academy of Sciences of the United States of America·G W SmallM E Phelps
May 17, 2000·Proceedings of the National Academy of Sciences of the United States of America·S I Rapoport
Sep 19, 2000·Journal of Magnetic Resonance Imaging : JMRI·C Z SimonsenC Gyldensted
Jul 13, 2001·Nature·N K LogothetisA Oeltermann
Oct 13, 2001·Journal of Cerebral Blood Flow and Metabolism : Official Journal of the International Society of Cerebral Blood Flow and Metabolism·D Attwell, S B Laughlin
Sep 24, 2002·Brain : a Journal of Neurology·Ruth M DixonA David Smith
Jan 14, 2003·Annual Review of Physiology·Douglas L RothmanRobert G Shulman
Mar 26, 2003·Magnetic Resonance in Medicine : Official Journal of the Society of Magnetic Resonance in Medicine·Ed X WuHaiying Tang
Sep 10, 2004·Journal of Cerebral Blood Flow and Metabolism : Official Journal of the International Society of Cerebral Blood Flow and Metabolism·Astrid NehligChang Dao Lam
Nov 17, 2004·Genes, Brain, and Behavior·Q J YanR P Bauchwitz
Dec 14, 2004·NeuroImage·Egill RostrupOlaf B Paulson
Dec 22, 2004·Nature Reviews. Neuroscience·Martin Lauritzen
Feb 15, 2005·Magnetic Resonance Imaging·Nikos K Logothetis, Josef Pfeuffer
Feb 15, 2005·Cell·Brant M Weinstein
Dec 21, 2005·The Journal of Cell Biology·Ji-Yeon ShinXiao-Jiang Li
Jan 4, 2006·NeuroRx : the Journal of the American Society for Experimental NeuroTherapeutics·Jia Yi LiPatrik Brundin
Jan 25, 2006·Journal of Neuroscience Methods·Annelyn Torres-ReverónDiana L Dow-Edwards
Mar 23, 2006·NMR in Biomedicine·Herman MorenoScott Small
Dec 16, 2006·Current Alzheimer Research·William Wu, Scott A Small
Feb 15, 2007·Proceedings of the National Academy of Sciences of the United States of America·William J PowersJoel S Perlmutter
Jun 15, 2007·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Emmanuelle ChaigneauSerge Charpak
Aug 19, 2007·Journal of the International Neuropsychological Society : JINS·Janice L ZimbelmanStephen M Rao
Oct 19, 2007·NeuroImage·Jason P LerchR Mark Henkelman
Jun 11, 2009·Journal of Cerebral Blood Flow and Metabolism : Official Journal of the International Society of Cerebral Blood Flow and Metabolism·Henning Piilgaard, Martin Lauritzen
Jul 3, 2009·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Richard KovácsOliver Kann
Aug 21, 2009·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Damian M CummingsMichael S Levine
Sep 4, 2009·Journal of Biomedical Optics·Theodore J HuppertDavid A Boas
Aug 11, 2010·Proceedings of the National Academy of Sciences of the United States of America·Peifang TianAnna Devor
Aug 20, 2010·Radiology·David S KarowUNKNOWN Alzheimer's Disease Neuroimaging Initiative

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Citations

Aug 30, 2013·The Journal of Clinical Investigation·Chris C TangDavid Eidelberg
May 22, 2013·Brain Structure & Function·Jeremy F P UllmannDavid C Reutens
Jan 24, 2014·Human Molecular Genetics·Angelica VittoriUNKNOWN REGISTRY investigators of the European Huntington's Disease Network
Jun 21, 2015·Expert Review of Neurotherapeutics·Cristina CarvalhoPaula I Moreira
Jun 19, 2014·Journal of Cerebral Blood Flow and Metabolism : Official Journal of the International Society of Cerebral Blood Flow and Metabolism·Lydie BoussicaultGilles Bonvento
Apr 22, 2015·Nature Reviews. Neuroscience·Ivan SolteszMasahiko Watanabe
Jan 1, 2016·PloS One·Adrián G Sandoval-HernándezGonzalo Arboleda
Aug 4, 2016·Journal of Cerebral Blood Flow and Metabolism : Official Journal of the International Society of Cerebral Blood Flow and Metabolism·Johanna A Gutiérrez-VargasGloria P Cardona-Gómez
Apr 25, 2015·Biochimica Et Biophysica Acta·Solomon T GizawShin-Ichiro Nishimura
Nov 11, 2017·Journal of Huntington's Disease·Janet M Dubinsky
Feb 28, 2014·Movement Disorders : Official Journal of the Movement Disorder Society·Abhishek ChandraM Flint Beal
Mar 7, 2019·Movement Disorders : Official Journal of the Movement Disorder Society·Yi-Ting HsuYijuang Chern
Jun 19, 2019·Journal of Cerebral Blood Flow and Metabolism : Official Journal of the International Society of Cerebral Blood Flow and Metabolism·Max I BogoradPeter C Searson
Jul 13, 2013·Movement Disorders : Official Journal of the Movement Disorder Society·Jun HuaChristopher A Ross
Aug 19, 2020·Journal of Cerebral Blood Flow and Metabolism : Official Journal of the International Society of Cerebral Blood Flow and Metabolism·Peter KlinkmuellerJun Hua
Dec 7, 2018·Open Biology·Yi-Ting HsuYijuang Chern
May 20, 2016·Human Molecular Genetics·Song LouJanet M Dubinsky
Apr 29, 2015·Annals of Neurology·Han-Yun HsiaoYijuang Chern
Feb 23, 2020·Magnetic Resonance in Medicine : Official Journal of the Society of Magnetic Resonance in Medicine·Philipp BackhausCornelius Faber
Apr 1, 2021·Neurotherapeutics : the Journal of the American Society for Experimental NeuroTherapeutics·Danielle A SimmonsFrank M Longo
Sep 17, 2013·Experimental Neurology·Chien-Yuan LinChen Chang
May 6, 2021·Proceedings of the National Academy of Sciences of the United States of America·Shaohong PengHo Yin Edwin Chan
May 15, 2021·Frontiers in Molecular Neuroscience·Tobias EngelMiguel Diaz-Hernandez
May 28, 2021·Brain : a Journal of Neurology·Hongshuai LiuWenzhen Duan

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