|Former Common name||B6D2-TgN(hAndR/CAGn)/97Q-7-8-917|
|ES Cell line|
|Strain development||Developer and Depositor: Hiroaki Adachi, University of Occupational and Environmental Health, Japan. Masahisa Katsuno, Nagoya University. The mice was generated by injecting AR-97Q construct into BDF1 fertilized eggs. The AR-97Q construct consisted of Chicken-actin promoter and human AR fragment harboring 97 CAGs. The founder Tg mice were backcrossed to C57BL/6J.|
|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|
|Examination Date / Room / Rack|
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)
|Donor DNA||CAG promoter (CMV-IE enhancer, chicken beta-actin promoter, rabbit beta-globin genomic DNA), human androgen receptor cDNA|
|Specific Term and Conditions||The RECIPIENT of BIOLOGICAL RESOURCE shall obtain a prior written consent on use of it from the DEPOSITOR. In publishing the research results obtained by use of the BIOLOGICAL RESOURCE, a citation of the following literature(s) designated by the DEPOSITOR is requested. Neuron, 35, 843-854 (2002). In publishing the research results to be obtained by use of the BIOLOGICAL RESOURCE, an acknowledgment to the DEPOSITOR is requested. RECIPIENT must contact the DEPOSITOR in the case of application for any patents or commercial use based on the results from the use of the BIOLOGICAL RESOURCE.|
|Depositor||Gen Sobue (Nagoya University)|
|Strain Status||Frozen embryos|
|Strain Availability||Recovered litters from cryopreserved embryos (2 to 4 months)|
Cryopreserved sperm (within 1 month)
Cryopreserved embryos (within 1 month)
|Additional Info.||Necessary documents for ordering:|
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