|Former Common name||B6D2-TgN(hAndR/CAGn)/97Q-7-8-917|
|H-2 Haplotype||No Data|
|Strain development||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.|
|Health Report||No Data|
|Promoter||CAG promoter (CMV-IE enhancer, chicken beta-actin promoter, rabbit beta-globin genomic DNA)|
|Symbol name||androgen receptor (human)|
|Common name||androgen receptor|
|Symbol description||No Data|
|References||Neuron. 2002 Aug 29;35(5):843-54.
|Research applications||No Data|
|Specific Term and Conditions||The following terms and conditions will be requested by 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.
Prior to requesting the BIOLOGICAL RESOURCE, the RECIPIENT must obtain approval form the DEPOSITOR using the Approval Form. 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.
|2||Genotyping protocol <PCR>|
|Depositor||Sobue, Gen (Nagoya University Graduate School of Medicine)|
|Strain Status /
|Testosterone reduction prevents phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy.||Neuron35: 843-854 (2002).(12372280)|
|Katsuno M, Adachi H, Kume A, Li M, Nakagomi Y, Niwa H, Sang C, 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: 2203-2211 (2003).(12657679)|
|Adachi H, Katsuno M, Minamiyama M, Sang C, Pagoulatos G, Angelidis C, Kusakabe M, Yoshiki A, Kobayashi Y, Doyu M, Sobue G.|
|Hsp105alpha suppresses the aggregation of truncated androgen receptor with expanded CAG repeats and cell toxicity.||J. Biol. Chem.278: 25143-25150 (2003).(12714591)|
|Ishihara K, Yamagishi N, Saito Y, Adachi H, Kobayashi Y, Sobue G, Ohtsuka K, Hatayama T.|
|Leuprorelin rescues polyglutamine-dependent phenotypes in a transgenic mouse model of spinal and bulbar muscular atrophy.||Nat. Med.9: 768-773 (2003).(12754502)|
|Katsuno M, Adachi H, Doyu M, Minamiyama M, Sang C, Kobayashi Y, Inukai A, Sobue G.|
|Transgenic mouse models of spinal and bulbar muscular atrophy (SBMA).||Cytogenet Genome Res.100(1-4): 243-51 (2003).(14526186)|
|Katsuno M, Adachi H, Inukai A, Sobue G.|
|Sodium butyrate ameliorates phenotypic expression in a transgenic mouse model of spinal and bulbar muscular atrophy.||Hum. Mol. Genet.13: 1183-1192 (2004).(15102712)|
|Minamiyama M, Katsuno M, Adachi H, Waza M, Sang C, Kobayashi Y, Tanaka F, Doyu M, Inukai A, 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, Tanaka F, Sobue G.|
|Widespread nuclear and cytoplasmic accumulation of mutant androgen receptor in SBMA patients.||Brain128: 659-670 (2005).(15659427)|
|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.|