Elucidation of the pathogenesis of creatine metabolism dysregulation in spinal and bulbar muscular atrophy (SBMA) and clinical trial of oral creatine for patients with SBMA
Project/Area Number |
26670440
|
Research Category |
Grant-in-Aid for Challenging Exploratory Research
|
Allocation Type | Multi-year Fund |
Research Field |
Neurology
|
Research Institution | Nagoya University |
Principal Investigator |
KASTUNO Masahisa 名古屋大学, 医学(系)研究科(研究院), 准教授 (50402566)
|
Co-Investigator(Kenkyū-buntansha) |
SOBUE Gen 名古屋大学, 医学系研究科, 教授 (20148315)
|
Project Period (FY) |
2014-04-01 – 2015-03-31
|
Project Status |
Completed (Fiscal Year 2014)
|
Budget Amount *help |
¥3,640,000 (Direct Cost: ¥2,800,000、Indirect Cost: ¥840,000)
Fiscal Year 2014: ¥3,640,000 (Direct Cost: ¥2,800,000、Indirect Cost: ¥840,000)
|
Keywords | 球脊髄性筋萎縮症 / 神経変性疾患 / 運動ニューロン / 骨格筋 / クレアチン |
Outline of Final Research Achievements |
Serum creatinine (Cr) levels decrease with disease progression of spinal and bulbar muscular atrophy (SBMA). To elucidate the pathogenesis of creatine-Cr metabolism dysregulation in SBMA, we included subjects with SBMA (n = 52) to assess motor function using functional parameters and measure muscle mass with dual-energy X-ray absorptiometry (DXA). We also examined the intra-muscular creatine concentration and analyzed the expression levels of creatine transporter in skeletal muscle using immunoblotting. Serum Cr levels correlated well with the functional parameters such as ALSFRS-R in SBMA. Both serum Cr and muscular creatine levels were lower in SBMA than in ALS, even though these groups had similar ALST. Furthermore, the expression levels of muscular creatine transporter were decreased in SBMA compared with ALS, providing a molecular basis for the decreased intra-muscular creatine in SBMA.
|
Report
(2 results)
Research Products
(11 results)
-
[Journal Article] Pioglitazone suppresses neuronal and muscular degeneration caused by polyglutamine-expanded androgen receptors.2015
Author(s)
Iida M, Katsuno M, Nakatsuji H, Adachi H, Kondo N, Miyazaki Y, Tohnai G, Ikenaka K, Watanabe H, Yamamoto M, Kishida K, Sobue G
-
Journal Title
Hum Mol Genet.
Volume: 24
Issue: 2
Pages: 314-329
DOI
Related Report
Peer Reviewed / Open Access / Acknowledgement Compliant
-
[Journal Article] Paeoniflorin eliminates a mutant AR via NF-YA-dependent proteolysis in spinal and bulbar muscular atrophy.2014
Author(s)
Tohnai G, Adachi H, Katsuno M, Doi H, Matsumoto S, Kondo N, Miyazaki Y, Iida M, Nakatsuji H, Qiang Q, Ding Y, Watanabe H, Yamamoto M, Ohtsuka K, Sobue G.
-
Journal Title
Hum Mol Genet.
Volume: 23
Issue: 13
Pages: 3552-3565
DOI
Related Report
Peer Reviewed / Open Access
-
[Journal Article] Brugada syndrome in spinal and bulbar muscular atrophy.2014
Author(s)
Araki A, Katsuno M, Suzuki K, Banno H, Suga N, Hashizume A, Mano T, Hijikata Y, Nakatsuji H, Watanabe H, Yamamoto M, Makiyama T, Ohno S, Fukuyama M, Morimoto S, Horie M, Sobue G
-
Journal Title
Neurology
Volume: 82
Issue: 20
Pages: 1813-1821
DOI
Related Report
Peer Reviewed / Acknowledgement Compliant
-
[Journal Article] Anti-androgen flutamide protects male mice from androgen-dependent toxicity in three models of spinal bulbar muscular atrophy2014
Author(s)
Renier KJ, Troxell-Smith SM, Johansen JA, Katsuno M, Adachi H, Sobue G, Chua JP, Sun Kim H, Lieberman AP, Breedlove SM, Jordan CL
-
Journal Title
Endocrinology.
Volume: 155
Issue: 7
Pages: 2624-2634
DOI
Related Report
Peer Reviewed
-
-
-
[Presentation] 運動ニューロン疾患における耐糖能異常の解析.2014
Author(s)
荒木周, 中辻秀朗, 勝野雅央, 鈴木啓介, 坂野晴彦, 須賀徳明, 橋詰淳, 土方靖浩, 祖父江 元.
Organizer
第55回日本神経学会学術大会
Place of Presentation
福岡県福岡市(福岡国際会議場)
Year and Date
2014-05-21 – 2014-05-24
Related Report
-
-
-
-