Brain Derived Neurotrophic Factor (BDNF) Delays Onset of Pathogenesis in Transgenic Mouse Model of Spinocerebellar Ataxia Type 1 (SCA1)

Frontiers in Cellular Neuroscience
Aaron MellesmoenMarija Cvetanovic


Spinocerebellar ataxia type 1 (SCA1) is a fatal neurodegenerative disease caused by an abnormal expansion of CAG repeats in the Ataxin-1 (ATXN1) gene and characterized by motor deficits and cerebellar neurodegeneration. Even though mutant ATXN1 is expressed from an early age, disease onset usually occurs in patient's mid-thirties, indicating the presence of compensatory factors that limit the toxic effects of mutant ATXN1 early in disease. Brain derived neurotrophic factor (BDNF) is a growth factor known to be important for the survival and function of cerebellar neurons. Using gene expression analysis, we observed altered BDNF expression in the cerebella of Purkinje neuron specific transgenic mouse model of SCA1, ATXN1[82Q] mice, with increased expression during the early stage and decreased expression in the late stage of disease. We therefore investigated the potentially protective role of BDNF in early stage SCA1 through intraventricular delivery of BDNF via ALZET osmotic pumps. Extrinsic BDNF delivery delayed onset of motor deficits and Purkinje neuron pathology in ATXN1[82Q] mice supporting its use as a novel therapeutic for SCA1.


Nov 30, 1999·Nature·K W KafitzA Konnerth
May 29, 2000·Brain Research·I FerrerT Ribalta
Oct 8, 2004·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Tao ZuHarry T Orr
May 20, 2006·Proceedings of the National Academy of Sciences of the United States of America·Kazuharu FurutaniMasamitsu Iino
Mar 24, 2007·Progress in Neurobiology·Chiara Zuccato, Elena Cattaneo
Apr 10, 2007·Annual Review of Neuroscience·Harry T Orr, Huda Y Zoghbi
Oct 18, 2007·Brain Cell Biology·Laurens W J BosmanArthur Konnerth
Nov 28, 2007·Journal of Neuroimmune Pharmacology : the Official Journal of the Society on NeuroImmune Pharmacology·Ramendra N SahaKalipada Pahan
Feb 27, 2008·Brain : a Journal of Neurology·Melina L WillsonRachel M Sherrard
Oct 30, 2008·The Journal of Biological Chemistry·Huda Y Zoghbi, Harry T Orr
Dec 3, 2009·The Journal of Cell Biology·Hristelina IlievaDon W Cleveland
Feb 24, 2010·Nature Reviews. Genetics·Albert R La Spada, J Paul Taylor
Aug 19, 2011·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Raphael HourezSerge N Schiffmann
Sep 9, 2011·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Justin A BarnesTimothy J Ebner
Mar 8, 2012·Neuropathology : Official Journal of the Japanese Society of Neuropathology·Makoto TakahashiHidehiro Mizusawa
Jul 13, 2012·The Neuroscientist : a Review Journal Bringing Neurobiology, Neurology and Psychiatry·Stefka Mincheva-Tasheva, Rosa M Soler
Mar 29, 2013·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Blake A EbnerHarry T Orr
May 8, 2013·Molecular Neurobiology·Yan-Yan HeJian-Jun Wang
May 28, 2013·Lancet Neurology·Elsdon Storey
Apr 4, 2014·Nature·Jeremy A MillerEd S Lein
Jun 26, 2014·Nature Reviews. Immunology·Michael T HenekaEicke Latz
Aug 14, 2015·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·James M Dell'OrcoVikram G Shakkottai
Dec 17, 2015·Acta Neuropathologica·Milos PeknyAlexei Verkhratsky
Aug 5, 2017·Nature Communications·Myeongjeong ChooMasanobu Kano
Sep 1, 2017·Nature Reviews. Neuroscience·Henry L PaulsonHarry T Orr

❮ Previous
Next ❯


Sep 24, 2020·Expert Opinion on Therapeutic Targets·Andreia Neves-CarvalhoPatrícia Maciel
Oct 17, 2019·Frontiers in Cellular Neuroscience·Dominik R GabrychMichael A Silverman
Mar 15, 2020·Journal of Clinical Medicine·Valentina Cerrato
Dec 6, 2020·International Journal of Molecular Sciences·Carrie SheelerMarija Cvetanovic
Feb 8, 2020·Current Neuropharmacology·Shareen Singh, Thakur Gurjeet Singh
May 22, 2021·Annals of Clinical and Translational Neurology·Sarah M BrookerPuneet Opal
May 21, 2021·Movement Disorders : Official Journal of the Movement Disorder Society·Chandrakanth Reddy Edamakanti, Puneet Opal

❮ Previous
Next ❯

Methods Mentioned


Software Mentioned


Related Concepts

Related Feeds

Brain Injury & Trauma

brain injury after impact to the head is due to both immediate mechanical effects and delayed responses of neural tissues.