RBRC00344

Information1

Image
BRC No.RBRC00344
TypeTransgeneCartagena
SpeciesMus musculus
Strain nameB6D2-Tg(CAG-AR*97Q)7-8Sobu
Former Common nameB6D2-TgN(hAndR/CAGn)/97Q-7-8-917
H-2 Haplotype
ES Cell line
Background strainJcl:B6D2F1
Appearance
Strain developmentThe 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 descriptionSpinal 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 maintenanceSurvival and reproduction are reduced by extensive backcross to B6. Maintenance in the BDF1 background may improve the survival of the Tg.
ReferencesNeuron, 35, 843-854 (2002). 12372280

Health Report

Examination Date / Room / RackNo Data

Gene

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

Information2

Donor DNACAG promoter (CMV-IE enhancer, chicken beta-actin promoter, rabbit beta-globin genomic DNA), human androgen receptor cDNA
Research application
Specific Term and ConditionsIn 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.
DepositorGen Sobue (Nagoya University)
Strain Statusan icon for Frozen embryosFrozen embryos
an icon for Frozen spermFrozen sperm
Strain AvailabilityRecovered litters from cryopreserved embryos (2 to 4 months)
Cryopreserved sperm (within 1 month)
Cryopreserved embryos (within 1 month)
Additional Info.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 128 659-670(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 1183-1192(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 25143-25150(2003) 12714591

Katsuno M, Adachi H, Tanaka F, Sobue G.
Spinal and bulbar muscular atrophy: ligand-dependent pathogenesis and therapeutic perspectives.
J. Mol. Med. 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 2203-2211(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. 9 768-773(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 35 843-854(2002) 12372280