Reduced brain-derived neurotrophic factor (BDNF) mRNA expression and presence of BDNF-immunoreactive granules in the spinocerebellar ataxia type 6 (SCA6) cerebellum

Neuropathology : Official Journal of the Japanese Society of Neuropathology
Makoto TakahashiHidehiro Mizusawa


Spinocerebellar ataxia type 6 (SCA6) is an autosomal-dominant neurodegenerative disorder caused by a small expansion of tri-nucleotide (CAG) repeat encoding polyglutamine (polyQ) in the gene for α(1A) voltage-dependent calcium channel (Ca(v) 2.1). Thus, this disease is one of the nine neurodegenerative disorders called polyQ diseases. The Purkinje cell predominant neuronal loss is the characteristic neuropathology of SCA6, and a 75-kDa carboxy-terminal fragment (CTF) of Ca(v) 2.1 containing polyQ, which remains soluble in normal brains, becomes insoluble in the cytoplasm of SCA6 Purkinje cells. Because the suppression of the brain-derived neurotrophic factor (BDNF) expression is a potentially momentous phenomenon in many other polyQ diseases, we implemented BDNF expression analysis in SCA6 human cerebellum using quantitative RT-PCR for the BDNF mRNA, and by immunohistochemistry for the BDNF protein. We observed significantly reduced BDNF mRNA levels in SCA6 cerebellum (n = 3) compared to controls (n = 6) (Mann-Whitney U-test, P = 0.0201). On immunohistochemistry, BDNF protein was only weakly stained in control cerebellum. On the other hand, we found numerous BDNF-immunoreactive granules in dendrites of SCA6 Purkinje cells. We d...Continue Reading


Jun 17, 1999·Journal of Neurology, Neurosurgery, and Psychiatry·Kinya IshikawaH Mizusawa
Aug 6, 1999·Ryōikibetsu shōkōgun shirīzu·Hidehiro Mizusawa
May 24, 2000·Proceedings of the National Academy of Sciences of the United States of America·J S SteffanL M Thompson
May 29, 2000·Brain Research·I FerrerT Ribalta
Aug 25, 2000·Human Molecular Genetics·A McCampbellK H Fischbeck
Oct 14, 2000·Experimental Neurology·D W HowellsG A Donnan
Jun 16, 2001·Science·Chiara ZuccatoElena Cattaneo
Jun 27, 2001·Neurology·Kinya IshikawaHidehiro Mizusawa
Sep 15, 2004·Journal of Human Genetics·Hiroki TakahashiHidehiro Mizusawa
Nov 3, 2004·Growth Factors·Devin K Binder, Helen E Scharfman
Jun 28, 2005·Biochemical and Biophysical Research Communications·Toshihide MiyashitaMasaaki Tsuda
Mar 3, 2007·Seminars in Pediatric Neurology·Richard E Lutz
Mar 24, 2007·Progress in Neurobiology·Chiara Zuccato, Elena Cattaneo
Apr 10, 2007·Annual Review of Neuroscience·Harry T Orr, Huda Y Zoghbi
Feb 8, 2008·Genes, Brain, and Behavior·K SchindowskiL Buée
Jan 22, 2009·Molecular Neurobiology·Nicola KuczewskiJean-Luc Gaiarsa
Nov 13, 2009·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Naoto MatsudaMu-Ming Poo
Aug 19, 2011·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Raphael HourezSerge N Schiffmann

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