B6;129S6-Gt(ROSA)26Sortm1(TARDBP*M337V/Ypet)Tlbt/JH
| Status | Available to order |
| EMMA ID | EM:15094 |
| International strain name | B6;129S6-Gt(ROSA)26Sortm1(TARDBP*M337V/Ypet)Tlbt/JH |
| Alternative name | TDP-43 M337V (TDPC) |
| Strain type | Targeted Mutant Strains : Knock-in |
| Allele/Transgene symbol | Gt(ROSA)26Sortm1(TARDBP*M337V/Ypet)Tlbt, |
| Gene/Transgene symbol | Gt(ROSA)26Sor |
Information from provider
| Provider | Kevin Talbot |
| Provider affiliation | Nuffield Department of Clinical Neurosciences, University of Oxford |
| Genetic information | This mouse expresses a single copy of a bacterial artificial chromosome (BAC) construct carrying the entire genomic locus of the human TARDBP (TDP-43) gene carrying the M337V mutation inserted into the mouse endogenous Gt(ROSA)26Sor locus. Human mutant TDP-43 is expressed at low levels on a background of mouse endogenous TDP-43. |
| Phenotypic information | Homozygous:Not viable.Heterozygous:Low-level stable expression of a single copy of human mutant TDP-43 M337V in mice leads to slowly progressive weakness and reduced survival. Abnormal stress granule assembly is associated with the amyotrophic lateral sclerosis (ALS)-causing TDP-43 M337V mutation. |
| Breeding history | This mouse line has 19 generations of cross-breeding. |
| References |
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| Homozygous fertile | not known |
| Homozygous viable | no |
| Homozygous matings required | no |
| Immunocompromised | not known |
Information from EMMA
| Archiving centre | Mary Lyon Centre at MRC Harwell, Oxford, United Kingdom |
Literature references
- Single-copy expression of an amyotrophic lateral sclerosis-linked TDP-43 mutation (M337V) in BAC transgenic mice leads to altered stress granule dynamics and progressive motor dysfunction.;Gordon David, Dafinca Ruxandra, Scaber Jakub, Alegre-Abarrategui Javier, Farrimond Lucy, Scott Connor, Biggs Daniel, Kent Louisa, Oliver Peter L, Davies Benjamin, Ansorge Olaf, Wade-Martins Richard, Talbot Kevin, ;2019;Neurobiology of disease;121;148-162; 30290270