B6.Cg-Prnp^tm1Cwe Tg(PrnpM128D177N)FFI-10Rchi/Cnrm

Status	Available to order
EMMA ID	EM:09930
International strain name	B6.Cg-Prnp^tm1Cwe Tg(PrnpM128D177N)FFI-10Rchi/Cnrm
Alternative name	Tg(FFI-10+/+)/Prnp0/0
Strain type	Targeted Mutant Strains : Knock-out
Allele/Transgene symbol	Prnp^tm1Cwe,
Gene/Transgene symbol	Prnp

Information from provider

Provider	Roberto Chiesa
Provider affiliation	Neuroscience, Istituto di Ricerche Farmacologiche Mario Negri
Genetic information	Transgenic mice expressing mouse prion protein (moPrP) carrying the D177N substitution that in humans is linked to fatal familial insomnia, under the control of the moPrP gene (Prnp) promoter.
Phenotypic information	Homozygous: Neurological disease with sleep abnormalities, cognitive and motor dysfunction. Neurological signs become overt at approximately 300 days and mice reach a terminal stage at approximately 700 days (Bouybayoune et al., PLoS Pathog 11(4): e1004796, 2015). Heterozygous: Mild neurological signs. Mice live as long as nontransgenic controls (Bouybayoune et al., PLoS Pathog 11(4): e1004796, 2015).
Breeding history	The Tg(FFI+/-)/Prnp+/+ founder was backcrossed with C57BL/6J/Prnp0/0 mice (EMMA strain EM:01723). After backcrossing for 10 generations, Tg(FFI+/+)Prnp0/0 mice were intercrossed to produce Tg(FFI+/+)/Prnp0/0 mice. Homozygous mice are maintained by intercrossing.
References	Transgenic fatal familial insomnia mice indicate prion infectivity-independent mechanisms of pathogenesis and phenotypic expression of disease.;Bouybayoune Ihssane, Mantovani Susanna, Del Gallo Federico, Bertani Ilaria, Restelli Elena, Comerio Liliana, Tapella Laura, Baracchi Francesca, Fernández-Borges Natalia, Mangieri Michela, Bisighini Cinzia, Beznoussenko Galina V, Paladini Alessandra, Balducci Claudia, Micotti Edoardo, Forloni Gianluigi, Castilla Joaquín, Fiordaliso Fabio, Tagliavini Fabrizio, Imeri Luca, Chiesa Roberto, ;2015;PLoS pathogens;11;e1004796; 25880443
Homozygous fertile	yes
Homozygous viable	yes
Homozygous matings required	no
Immunocompromised	not known

Information from EMMA

Archiving centre	CNR, Consiglio Nazionale delle Ricerche, Monterotondo, Italy

Disease and phenotype information

Orphanet associated rare diseases, based on orthologous gene matching

Inherited Creutzfeldt-Jakob disease / Orphanet_282166
Familial Alzheimer-like prion disease / Orphanet_280397
Huntington disease-like 1 / Orphanet_157941
PrP systemic amyloidosis / Orphanet_397606
Fatal familial insomnia / Orphanet_466
Gerstmann-Straussler-Scheinker syndrome / Orphanet_356
Sporadic fatal insomnia / Orphanet_586130

MGI phenotypes (allele matching)

reduced long term potentiation / MGI
abnormal CNS synaptic transmission / MGI
abnormal inhibitory postsynaptic potential / MGI
abnormal inhibitory postsynaptic currents / MGI
behavior/neurological phenotype / MGI
decreased brain copper level / MGI
tremors / MGI
abnormal cerebellum morphology / MGI
decreased Purkinje cell number / MGI

Literature references

Transgenic fatal familial insomnia mice indicate prion infectivity-independent mechanisms of pathogenesis and phenotypic expression of disease.;Bouybayoune Ihssane, Mantovani Susanna, Del Gallo Federico, Bertani Ilaria, Restelli Elena, Comerio Liliana, Tapella Laura, Baracchi Francesca, Fernández-Borges Natalia, Mangieri Michela, Bisighini Cinzia, Beznoussenko Galina V, Paladini Alessandra, Balducci Claudia, Micotti Edoardo, Forloni Gianluigi, Castilla Joaquín, Fiordaliso Fabio, Tagliavini Fabrizio, Imeri Luca, Chiesa Roberto, ;2015;PLoS pathogens;11;e1004796; 25880443

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Availabilities

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

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

Due to the dynamic nature of our processes strain availability may change at short notice. The local repository manager will advise you in these circumstances.

^* In addition users have to cover all the shipping costs (including the cost for returning dry-shippers, where applicable).

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Practical information

Genotyping protocol

Example health report
(Current health report will be provided later)

Material Transfer Agreement (MTA)
For this strain no provider MTA is needed. Distribution is based on the EMMA conditions only.

EMMA conditions
Legally binding conditions for the transfer

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B6.Cg-Prnptm1Cwe Tg(Prnp*M128*D177N)FFI-10Rchi/Cnrm