STOCK Serpinf2tm1Hrl Tg(Alb-TTR*S52P)N4Psim/H

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EMMA IDEM:13232
Citation informationRRID:IMSR_EM:13232 

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International strain nameSTOCK Serpinf2tm1Hrl Tg(Alb-TTR*S52P)N4Psim/H
Alternative nameSTOCK Tg(Alb-TTR*S52P)N4Psim Serpinf2tm1Hrl
Strain typeTargeted Mutant Strains : Knock-out
Allele/Transgene symbolSerpinf2tm1Hrl, Tg(Alb-TTR*S52P)N4Psim
Gene/Transgene symbolSerpinf2, Tg(Alb-TTR*S52P)N4Psim

Information from provider

ProviderPaul Simons
Provider affiliationCentre for Amyloidosis & Acute Phase Proteins, Division of Medicine, UCL
Additional ownerProf. Dr. Lijnen H.R., Center for Molecular and Vascular Biology, Department of Cardiovascular Sciences, Leuven, Belgium
Genetic informationTransgenic for albumin enhancer/promoter-driven human transthyretin S52P amyloidogenic variant (TTR-S52P) gene and knock-out for alpha-2 antiplasmin (Serpinf2).
Phenotypic informationHomozygous:
Phenotype of TTR-S52P transgenic homozygotes unknown.

Heterozygous:
Can be used to model human systemic transthyretin (ATTR) amyloidosis. Mice hemizygous for the TTR-S52P transgene express the S52P amyloidogenic variant of human TTR very abundantly (serum concentrations: 6.8 ± 0.8 mg/ml, males; 4.5 ± 1.0 mg/ml, females). TTR amyloid deposition can be seeded by i.v. injection of ex vivo ATTR amyloid. No TTR amyloid deposition has been observed in the absence of seeding with ex vivo ATTR amyloid. Seeded ATTR amyloid deposition is accelerated by homozygous alpha2-antiplasmin (Serpinf2) knock-out. TTR transgenic mice are smaller than non-transgenic littermates.
Breeding historyTTR transgene introduced by pronuclear microinjection in C57BL/6 x CBA embryos, backcrossed to C57BL/6. Alpha-2 antiplasmin (Serpinf2) knock-out allele generated in 129/SvJ ES cells, backcrossed to C57BL/6. Number of generations unknown.
References
  • Plasmin activity promotes amyloid deposition in a transgenic model of human transthyretin amyloidosis.;Slamova Ivana, Adib Rozita, Ellmerich Stephan, Golos Michal R, Gilbertson Janet A, Botcher Nicola, Canetti Diana, Taylor Graham W, Rendell Nigel, Tennent Glenys A, Verona Guglielmo, Porcari Riccardo, Mangione P Patrizia, Gillmore Julian D, Pepys Mark B, Bellotti Vittorio, Hawkins Philip N, Al-Shawi Raya, Simons J Paul, ;2021;Nature communications;12;7112; 34876572
Homozygous fertilenot known
Homozygous viablenot known
Homozygous matings requiredno
Immunocompromisedno

Information from EMMA

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

Literature references

  • Plasmin activity promotes amyloid deposition in a transgenic model of human transthyretin amyloidosis.;Slamova Ivana, Adib Rozita, Ellmerich Stephan, Golos Michal R, Gilbertson Janet A, Botcher Nicola, Canetti Diana, Taylor Graham W, Rendell Nigel, Tennent Glenys A, Verona Guglielmo, Porcari Riccardo, Mangione P Patrizia, Gillmore Julian D, Pepys Mark B, Bellotti Vittorio, Hawkins Philip N, Al-Shawi Raya, Simons J Paul, ;2021;Nature communications;12;7112; 34876572

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