Strain Data Sheet
RBRC00344
Strain Information | |
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| Image |  |
| BRC No. | RBRC00344 |
| Type | Transgene |
| Species | Mus musculus |
| Strain name | B6D2-Tg(CAG-AR*97Q)7-8Sobu |
| Former Common name | B6D2-TgN(hAndR/CAGn)/97Q-7-8-917 |
| H-2 Haplotype | |
| ES Cell line | |
| Background strain | Jcl:B6D2F1 |
| Appearance | |
| Strain development | 開発及び寄託者：足立弘明（現 産業医科大学）・勝野雅央（名古屋大学） |
| Strain description | Spinal and bulbar muscular atrophy (SBMA) is a polyglutamine disease with expansion of a CAG repeat in the androgen receptor (AR) gene. This transgenic mice serves as a SBMA model carrying a full-length AR containing 97 CAGs. The mice showed progressive muscular atrophy and weakness. The mice also showed diffuse nuclear staining and nuclear inclusions. These phenotypes were significant in male transgenic mice, and rescued by castration. Female transgenic mice showed much limited phenotypes which became more pronounced by administration of testosterone. |
| Colony maintenance | Survival and reproduction are reduced by extensive backcross to B6. Maintenance in the BDF1 background may improve the survival of the Tg. |
| References | Testosterone reduction prevents phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy. Masahisa Katsuno, Hiroaki Adachi, Akito Kume, Mei Li, Yuji Nakagomi, Hisayoshi Niwa, Chen Sang, Yasushi Kobayashi, Manabu Doyu, Gen Sobue Neuron, 35, 843-854 (2002). 12372280 |
Health Report | |
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| Examination Date / Room / Rack | |
Gene | |||||||
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| Gene Symbol | Gene Name | Chr. | Allele Symbol | Allele Name | Common Names | Promoter | Diseases Related to This Gene |
| AR | androgen receptor (human) | UN | AR | CAG promoter (CMV-IE enhancer, chicken beta-actin promoter, rabbit beta-globin genomic DNA) | |||
Phenotype | |
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| Phenotype annotation from literatures by Mammalian phenotype ontology | more 13 phenotypes |
| Detailed phenotype data | |
Ordering Information | |
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| 供与核酸 | CAG promoter (CMV-IE enhancer, chicken beta-actin promoter, rabbit beta-globin genomic DNA), human androgen receptor cDNA |
| Research application | |
| 提供条件 | 利用者は事前に寄託者の提供承諾書を得る。研究成果の公表にあたって寄託者の指定する文献を引用する。Neuron, 35, 843-854 (2002). 研究成果の公表にあたって謝辞の表明を必要とする。利用者が本件リソースを使用して得られた研究成果に基づき特許等の申請、及び事業活動を行う場合は、寄託者と別途協議を行う。 |
| Depositor | 祖父江 元（名古屋大学） |
| Strain Status |  凍結胚 凍結精子 |
| Strain Availability | 凍結胚より作出したマウスを2~4ヶ月以内に提供可能 凍結精子を1ヶ月以内に提供可能 凍結胚を1ヶ月以内に提供可能 |
| Additional Info. | Necessary documents for ordering:
Genotyping protocol -PCR- |
BRC mice in Publications |
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Adachi H, Katsuno M, Minamiyama M, Waza M, Sang C, Nakagomi Y, Kobayashi Y, Tanaka F, Doyu M, Inukai A, Yoshida M, Hashizume Y, Sobue G. Widespread nuclear and cytoplasmic accumulation of mutant androgen receptor in SBMA patients. Brain 128(Pt 3) 659-70(2005) 15659427 |
Minamiyama M, Katsuno M, Adachi H, Waza M, Sang C, Kobayashi Y, Tanaka F, Doyu M, Inukai A, Sobue G. Sodium butyrate ameliorates phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy. Hum Mol Genet 13(11) 1183-92(2004) 15102712 |
Katsuno M, Adachi H, Tanaka F, Sobue G. Spinal and bulbar muscular atrophy: ligand-dependent pathogenesis and therapeutic perspectives. J Mol Med (Berl) 82(5) 298-307(2004) 15133611 |
Katsuno M, Adachi H, Inukai A, Sobue G. Transgenic mouse models of spinal and bulbar muscular atrophy (SBMA). Cytogenet Genome Res 100(1-4) 243-51(2003) 14526186 |
Ishihara K, Yamagishi N, Saito Y, Adachi H, Kobayashi Y, Sobue G, Ohtsuka K, Hatayama T. Hsp105alpha suppresses the aggregation of truncated androgen receptor with expanded CAG repeats and cell toxicity. J Biol Chem 278(27) 25143-50(2003) 12714591 |
Adachi H, Katsuno M, Minamiyama M, Sang C, Pagoulatos G, Angelidis C, Kusakabe M, Yoshiki A, Kobayashi Y, Doyu M, Sobue G. Heat shock protein 70 chaperone overexpression ameliorates phenotypes of the spinal and bulbar muscular atrophy transgenic mouse model by reducing nuclear-localized mutant androgen receptor protein. J Neurosci 23(6) 2203-11(2003) 12657679 |
Katsuno M, Adachi H, Doyu M, Minamiyama M, Sang C, Kobayashi Y, Inukai A, Sobue G. Leuprorelin rescues polyglutamine-dependent phenotypes in a transgenic mouse model of spinal and bulbar muscular atrophy. Nat Med 9(6) 768-73(2003) 12754502 |
Katsuno M, Adachi H, Kume A, Li M, Nakagomi Y, Niwa H, Sang C, Kobayashi Y, Doyu M, Sobue G. Testosterone reduction prevents phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy. Neuron 35(5) 843-54(2002) 12372280 |