B6.Cg-Tg(Thy1-SERPINI1*S52R)794Zbz/Biat
| Status | Available to order |
| EMMA ID | EM:11490 |
| Citation information | RRID:IMSR_EM:11490 Research Resource Identifiers (RRID) are persistent unique ID numbers assigned to help researchers cite key resources (e.g. antibodies, model organisms and software projects) in the biomedical literature to improve transparency and reproducibility in research. See https://www.rrids.org/ for more information. |
| International strain name | B6.Cg-Tg(Thy1-SERPINI1*S52R)794Zbz/Biat |
| Alternative name | Human S52R-Portland mutated neuroserpin |
| Strain type | Transgenic Strains |
| Allele/Transgene symbol | Tg(Thy1-SERPINI1*S52R)794Zbz |
| Gene/Transgene symbol | Tg(Thy1-SERPINI1*S52R)794Zbz |
Information from provider
| Provider | Thomas Rülicke |
| Provider affiliation | Biomedical Sciences, University of Veterinary Medicine |
| Genetic information | Transgenic mice overexpressing human neuroserpin with the S52R-Portland mutation under the control of the Thy1 promoter were generated by pronuclear injection into fertilized oocytes using an expression cassette derived from the murine Thy1 gene. The S52R-Portland mutation was introduced into the human neuroserpin cDNA using the polymerase chain reaction technique. The transgene was expressed in central nervous system neurons throughout postnatal life. The expression levels for transgenic mice with human Portland neuroserpin is 3.3 times [line Tg(NSS52R)794Zbz] above the endogenous neuroserpin level of wild-type mice. |
| Phenotypic information | Homozygous:unknownHeterozygous:Transgenic mice appeared healthy at birth, developed normally, and did not exhibit any obvious neurological or behavioral abnormalities during early adulthood. However, at 17 and 21 months of age the Portland line 794 mice began to exhibit severe neurological symptoms and behavioral abnormalities, reminiscent of those found in familial encephalopathy with neuroserpin inclusion bodies (FENIB) patients. They developed severe ataxia, moved slowly with staggering gait, were incapable of grasping a horizontal wire and climbing a grid of negative geotaxis, and exhibited a considerably greater passivity during handling. In addition, mice overexpressing Portland neuroserpin showed tremor and seizure-like episodes. |
| Breeding history | The transgenic lines were generated on a B6D2F2 hybrid background and backcrossed to C57BL/6J for at least 10 generations. |
| References | None available |
| Homozygous fertile | not known |
| Homozygous viable | not known |
| Homozygous matings required | no |
| Immunocompromised | not known |
Information from EMMA
| Archiving centre | University of Veterinary Medicine, Vienna, Austria |
| Animals used for archiving | heterozygous Other (please specify below) males |