B6J;129-Sod1tm1.1(SOD1)Emcf/H

Status

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EMMA IDEM:13075
Citation informationRRID:IMSR_EM:13075 

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International strain nameB6J;129-Sod1tm1.1(SOD1)Emcf/H
Alternative nameC57BL/6J-Sod1 tm1.1(SOD1)Emcf/H
Strain typeTargeted Mutant Strains : Knock-in
Allele/Transgene symbolSod1tm1.1(SOD1)Emcf
Gene/Transgene symbolSod1

Information from provider

ProviderElizabeth Fisher
Provider affiliationDepartment of Neuromuscular Diseases, UCL
Genetic informationC57BL/6J-Sod1tm1.1(SOD1)Emcf/H knock-in mice carry the human version of the Cu,Zn-superoxide dismutase-1 (SOD1) gene, which replaces the mouse Sod1 gene at the endogenous locus. The humanized region begins at the first codon in exon 1 of the Sod1 gene, and continues through to the end of the human SOD1 5’ UTR. Exons 4, 5 and the 5’ UTR of the humanized Sod1 gene have been floxed and duplicated to allow conditional expression of a second copy of exons 4 and 5. These mice were generated using homologous recombination in R1 ES cells.
Phenotypic informationHomozygous:
Homozygous mice are viable, fertile and produced at normal mendelian ratios. Homozygous mice have no overt phenotype (comprehensive phenotyping has not yet been conducted). The oldest homozygous C57BL/6J-Sod1/H mice are currently 8.5 months old.

Heterozygous:
Heterozygous mice are viable, fertile, and produced at normal mendelian ratios. Heterozygous mice have no overt phenotype (comprehensive phenotyping has not yet been conducted). The oldest heterozygous C57BL/6J-Sod1/H mice are currently 8.5 months old.
Breeding historyTargeting was performed in R1 ES cells, followed by injection into CD1 blastocysts. Chimeric offspring were crossed to C57BL/6J and founder C57BL/6J-Sod1tm1(SOD1)Emcf/H mice carrying the humanized allele were selected and crossed to C57BL/6J for one subsequent generation. C57BL/6J-Sod1tm1(SOD1)Emcf/H embryos were harvested from superovulated females, underwent IVF with C57BL/6J sperm and cytoplasmic injection of Flpo mRNA to remove the neomycin selection cassette. C57BL/6J-Sod1tm1.1(SOD1)Emcf/H mice carrying human SOD1 with the neomycin selection cassette excised were selected and backcrossed onto C57BL/6J for 2 generations.
References
  • Generation and analysis of innovative genomically humanized knockin SOD1, TARDBP (TDP-43), and FUS mouse models.;Devoy Anny, Price Georgia, De Giorgio Francesca, Bunton-Stasyshyn Rosie, Thompson David, Gasco Samanta, Allan Alasdair, Codner Gemma F, Nair Remya R, Tibbit Charlotte, McLeod Ross, Ali Zeinab, Noda Judith, Marrero-Gagliardi Alessandro, Brito-Armas José M, Williams Chloe, Öztürk Muhammet M, Simon Michelle, O'Neill Edward, Bryce-Smith Sam, Harrison Jackie, Atkins Gemma, Corrochano Silvia, Stewart Michelle, Gilthorpe Jonathan D, Teboul Lydia, Acevedo-Arozena Abraham, Fisher Elizabeth M C, Cunningham Thomas J, ;2021;iScience;24;103463; 34988393
Homozygous fertileyes
Homozygous viableyes
Homozygous matings requiredno
Immunocompromisedno

Information from EMMA

Archiving centreMary Lyon Centre at MRC Harwell, Oxford, United Kingdom

Disease and phenotype information

Orphanet associated rare diseases, based on orthologous gene matching

Literature references

  • Generation and analysis of innovative genomically humanized knockin SOD1, TARDBP (TDP-43), and FUS mouse models.;Devoy Anny, Price Georgia, De Giorgio Francesca, Bunton-Stasyshyn Rosie, Thompson David, Gasco Samanta, Allan Alasdair, Codner Gemma F, Nair Remya R, Tibbit Charlotte, McLeod Ross, Ali Zeinab, Noda Judith, Marrero-Gagliardi Alessandro, Brito-Armas José M, Williams Chloe, Öztürk Muhammet M, Simon Michelle, O'Neill Edward, Bryce-Smith Sam, Harrison Jackie, Atkins Gemma, Corrochano Silvia, Stewart Michelle, Gilthorpe Jonathan D, Teboul Lydia, Acevedo-Arozena Abraham, Fisher Elizabeth M C, Cunningham Thomas J, ;2021;iScience;24;103463; 34988393

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Requesting frozen sperm or embryos is generally advisable wherever possible, in order to minimise the shipment of live mice.

  • Frozen sperm. Delivered in 4 weeks (after paperwork in place). €1740*
  • Rederivation of mice from frozen stock, delivery time available upon request . €3880*
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