Strain Data Sheet

RBRC00344

update : 2024-03-19

Strain Information
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	Neuron, 35, 843-854 (2002). 12372280

Health Report
Examination Date / Room / Rack

Gene
Gene info	Gene symbolGene nameChr.Allele symbolAllele nameCommon namesPromoter ARandrogen receptor (human)UNARandrogen receptorCAG promoter (CMV-IE enhancer, chicken beta-actin promoter, rabbit beta-globin genomic DNA)

Ordering Information
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	条件を付加する。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: Approval form (Japanese / English) Order form (Japanese / English) Category I MTA: MTA for distribution with RIKEN BRC (Japanese / English) CAGGS MTA (English) Acceptance of responsibility for living modified organism (Japanese / English) Genotyping protocol -PCR-

BRC mice in Publications

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 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 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 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 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 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

BRC mice in Publications

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

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

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

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

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

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

Strain Information

Health Report

Gene

Ordering Information

BRC mice in Publications