Strain Data Sheet
Data update: Jul 19, 2019
Health Report   PCR Protocol   Thawing Method
embryos / sperm >
  Mouse Marking
RBRC No. RBRC05350  
Type TransgeneCartagena  
Species Mus musculus  
Strain name B6;B6D2-Tg(Thy1-BSCL2*N88S)1  
Former Common name No Data  
H-2 Haplotype No Data  
Background strain No Data  
 1 Appearance No Data  
Genotype No Data  
Strain development Developed by Daisuke Ito, School of Medicine, Keio University. A human seipin cDNA with the N88S mutation was inserted into the pTSC21k vector, which encodes the 8.1 kb murine Thy1.2 expression cassette. This construct was Not I digested to remove vector sequences, co-injected into the pronucleus of fertilized eggs from Slc:B6D2F1 strain mice. Founders were bred and backcrossed with C57BL6/J mice to establish transgenic lines.  
Strain description Heterozygosity for mutations (N88S L) in the seipin/BSCL2 is associated with the autosomal dominant motor neuron diseases, seipinopathy/seipin/BSCL2-related motor neuron diseases. A previous in vitro study suggested that ER stress is a key process in the pathogenesis of seipinopathies. Transgenic mouse line expressing the human N88S seipin mutant develop age-dependent motor phenotypes, which recapitulate key clinical features of seipinopathy. Remarkably, this phenotype was associated with ER stress in affected neuronal tissue. Therefore, this Tg mouse provides clues for understanding its relationship with ER stress and neurodegeneration, and could be a valid tool for the development of novel therapeutic strategies against ER stress-related diseases. N88S seipin Tg mice develop a progressive spastic motor deficit, reactive gliosis in the spinal cord and neurogenic muscular atrophy, recapitulating the symptomatic and pathological phenotype in patients of seipinopathy by the age of 28 weeks.  
Colony maintenance Carrier X Noncarrier [C57BL/6JJcl]  
Health Report
Gene Details
Promoter No Data  
 1 Symbol No Data  
Symbol name No Data  
Chromosome No Data  
Common name No Data  
Symbol description No Data  
Research applications No Data  
Specific Term and Conditions The following terms and conditions will be requested by the DEPOSITOR.
Prior to requesting the BIOLOGICAL RESOURCE, the RECIPIENT must obtain approval from the DEPOSITOR using the Approval Form.  
Additional information
 1 Mouse of the Month Dec 2011  
 2 Genotyping protocol <PCR>  
Depositor Ito, Daisuke (Keio University School of Medicine)  Ito, Daisuke 
Strain Status /
(Expected delivery)

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