Skip Navigation


Human Molecular Genetics Advance Access originally published online on April 21, 2004
This Article
Right arrow Full Text Freely available
Right arrow FREE Full Text (PDF) Freely available
Right arrow All Versions of this Article:
13/11/1183    most recent
ddh131v1
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in ISI Web of Science
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrow Search for citing articles in:
ISI Web of Science (51)
Right arrowRequest Permissions
Google Scholar
Right arrow Articles by Minamiyama, M.
Right arrow Articles by Sobue, G.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Minamiyama, M.
Right arrow Articles by Sobue, G.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

Human Molecular Genetics, 2004, Vol. 13, No. 11 1183-1192
DOI: 10.1093/hmg/ddh131
Human Molecular Genetics, Vol. 13, No. 11 © Oxford University Press 2004; all rights reserved

Sodium butyrate ameliorates phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy

Makoto Minamiyama, Masahisa Katsuno, Hiroaki Adachi, Masahiro Waza, Chen Sang, Yasushi Kobayashi, Fumiaki Tanaka, Manabu Doyu, Akira Inukai and Gen Sobue*

Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya 466-8550, Japan

Received February 23, 2004; Revised March 24, 2004; Accepted April 5, 2004

Spinal and bulbar muscular atrophy (SBMA) is an inherited motor neuron disease caused by the expansion of a polyglutamine (polyQ) tract within the androgen receptor. Unifying mechanisms have been implicated in the pathogenesis of polyQ-dependent neurodegenerative diseases including SBMA, Huntington disease and spinocerebellar ataxias. It has been suggested that mutant protein containing polyQ inhibits histone acetyltransferase activity, resulting in transcriptional dysfunction and subsequent neuronal dysfunction. Histone deacetylase (HDAC) inhibitors alleviate neurological phenotypes in fly and mouse models of polyQ disease, although the therapeutic effect is limited by the toxicity of these compounds. We studied the therapeutic effects of sodium butyrate (SB), an HDAC inhibitor, in a transgenic mouse model of SBMA. Oral administration of SB ameliorated neurological phenotypes as well as increased acetylation of nuclear histone in neural tissues. These therapeutic effects, however, were seen only within a narrow range of SB dosage. Our results indicate that SB is a possible therapeutic agent for SBMA and other polyQ diseases, although an appropriate dose should be determined for clinical application.

* To whom correspondence should be addressed at: Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8550, Japan. Tel: +81 527442385; Fax: +81 527442384; Email: sobueg{at}med.nagoya-u.ac.jp


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?


This article has been cited by other articles:


Home page
 Proc. Natl. Acad. Sci. USAHome page
R. B. Parmigiani, W. S. Xu, G. Venta-Perez, H. Erdjument-Bromage, M. Yaneva, P. Tempst, and P. A. Marks
HDAC6 is a specific deacetylase of peroxiredoxins and is involved in redox regulation
PNAS, July 15, 2008; 105(28): 9633 - 9638.
[Abstract] [Full Text] [PDF]

Home page
 BrainHome page
K. Suzuki, M. Katsuno, H. Banno, Y. Takeuchi, N. Atsuta, M. Ito, H. Watanabe, F. Yamashita, N. Hori, T. Nakamura, et al.
CAG repeat size correlates to electrophysiological motor and sensory phenotypes in SBMA
Brain, January 1, 2008; 131(1): 229 - 239.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
J. Shao and M. I. Diamond
Polyglutamine diseases: emerging concepts in pathogenesis and therapy
Hum. Mol. Genet., October 15, 2007; 16(R2): R115 - R123.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
H. Adachi, M. Waza, K. Tokui, M. Katsuno, M. Minamiyama, F. Tanaka, M. Doyu, and G. Sobue
CHIP Overexpression Reduces Mutant Androgen Receptor Protein and Ameliorates Phenotypes of the Spinal and Bulbar Muscular Atrophy Transgenic Mouse Model
J. Neurosci., May 9, 2007; 27(19): 5115 - 5126.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
M. Latouche, C. Lasbleiz, E. Martin, V. Monnier, T. Debeir, A. Mouatt-Prigent, M.-P. Muriel, L. Morel, M. Ruberg, A. Brice, et al.
A Conditional Pan-Neuronal Drosophila Model of Spinocerebellar Ataxia 7 with a Reversible Adult Phenotype Suitable for Identifying Modifier Genes
J. Neurosci., March 7, 2007; 27(10): 2483 - 2492.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
M. Katsuno, H. Adachi, M. Minamiyama, M. Waza, K. Tokui, H. Banno, K. Suzuki, Y. Onoda, F. Tanaka, M. Doyu, et al.
Reversible Disruption of Dynactin 1-Mediated Retrograde Axonal Transport in Polyglutamine-Induced Motor Neuron Degeneration.
J. Neurosci., November 22, 2006; 26(47): 12106 - 12117.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
E. Kontopoulos, J. D. Parvin, and M. B. Feany
{alpha}-synuclein acts in the nucleus to inhibit histone acetylation and promote neurotoxicity
Hum. Mol. Genet., October 15, 2006; 15(20): 3012 - 3023.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
T. F. Satterfield and L. J. Pallanck
Ataxin-2 and its Drosophila homolog, ATX2, physically assemble with polyribosomes
Hum. Mol. Genet., August 15, 2006; 15(16): 2523 - 2532.
[Abstract] [Full Text] [PDF]

Home page
 Genes Dev.Home page
B. E. Riley and H. T Orr
Polyglutamine neurodegenerative diseases and regulation of transcription: assembling the puzzle.
Genes & Dev., August 15, 2006; 20(16): 2183 - 2192.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
U. A. Desai, J. Pallos, A. A. K. Ma, B. R. Stockwell, L. M. Thompson, J. L. Marsh, and M. I. Diamond
Biologically active molecules that reduce polyglutamine aggregation and toxicity
Hum. Mol. Genet., July 1, 2006; 15(13): 2114 - 2124.
[Abstract] [Full Text] [PDF]

Home page
 BrainHome page
N. Atsuta, H. Watanabe, M. Ito, H. Banno, K. Suzuki, M. Katsuno, F. Tanaka, A. Tamakoshi, and G. Sobue
Natural history of spinal and bulbar muscular atrophy (SBMA): a study of 223 Japanese patients
Brain, June 1, 2006; 129(6): 1446 - 1455.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
M. Ying, R. Xu, X. Wu, H. Zhu, Y. Zhuang, M. Han, and T. Xu
Sodium Butyrate Ameliorates Histone Hypoacetylation and Neurodegenerative Phenotypes in a Mouse Model for DRPLA
J. Biol. Chem., May 5, 2006; 281(18): 12580 - 12586.
[Abstract] [Full Text] [PDF]

Home page
 Proc. Natl. Acad. Sci. USAHome page
M. Katsuno, C. Sang, H. Adachi, M. Minamiyama, M. Waza, F. Tanaka, M. Doyu, and G. Sobue
Pharmacological induction of heat-shock proteins alleviates polyglutamine-mediated motor neuron disease
PNAS, November 15, 2005; 102(46): 16801 - 16806.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
T. A. Bolger and T.-P. Yao
Intracellular Trafficking of Histone Deacetylase 4 Regulates Neuronal Cell Death
J. Neurosci., October 12, 2005; 25(41): 9544 - 9553.
[Abstract] [Full Text] [PDF]

Home page
 J Child NeurolHome page
D. Pleasure
New Treatments for Denervating Diseases
J Child Neurol, March 1, 2005; 20(3): 258 - 262.
[Abstract] [PDF]


Disclaimer:
Please note that abstracts for content published before 1996 were created through digital scanning and may therefore not exactly replicate the text of the original print issues. All efforts have been made to ensure accuracy, but the Publisher will not be held responsible for any remaining inaccuracies. If you require any further clarification, please contact our Customer Services Department.