B6.Cg-Prnp^tm1Cwe Tg(PrnpM128D177N)FFI-26Rchi/Cnrm

Status	Available to order
EMMA ID	EM:12861
International strain name	B6.Cg-Prnp^tm1Cwe Tg(PrnpM128D177N)FFI-26Rchi/Cnrm
Alternative name	Tg(FFI-26+/-)/Prnp0/0
Strain type	Targeted Mutant Strains : Knock-out
Allele/Transgene symbol	Prnp^tm1Cwe, Tg(PrnpM128D177N)FFI-26Rchi
Gene/Transgene symbol	Prnp, Tg(PrnpM128D177N)FFI-26Rchi

Information from provider

Provider	Roberto Chiesa
Provider affiliation	Neuroscience, Istituto di Ricerche Farmacologiche Mario Negri IRCCS
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 mouse Prnp (PrP) gene promoter.
Phenotypic information	Homozygous: Unknown. Heterozygous: Neurological disease with sleep abnormalities, cognitive and motor dysfunction. Neurological signs become overt at approximately 200 days and mice reach a terminal stage at approximately 440 days (Bouybayoune et al., PLoS Pathog 11(4): e1004796, 2015).
Breeding history	The Tg(FFI-26+/-)/Prnp+/+ founder (C57BL/6 x CBA) was backcrossed with C57BL/6J-backcrossed Prnp0/0 mice (EMMA strain EM:01723).
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	not known
Homozygous viable	not known
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

MGI phenotypes (gene matching)

impaired fertilization / MGI
abnormal spleen morphology / MGI
tremors / MGI
abnormal cerebral cortex morphology / MGI
abnormal hippocampus morphology / MGI
abnormal olfactory bulb morphology / MGI
abnormal thalamus morphology / MGI
abnormal cerebellum morphology / MGI
Purkinje cell degeneration / MGI
decreased Purkinje cell number / MGI
abnormal cerebellar molecular layer / MGI
thin cerebellar molecular layer / MGI
abnormal retina morphology / MGI
ataxia / MGI
hypoactivity / MGI
impaired coordination / MGI
reduced long term potentiation / MGI
abnormal sleep pattern / MGI
abnormal body temperature homeostasis / MGI
male infertility / MGI
premature death / MGI
abnormal muscle physiology / MGI
abnormal brain morphology / MGI
no abnormal phenotype detected / MGI
gliosis / MGI
abnormal CNS synaptic transmission / MGI
neurodegeneration / MGI
spongiform encephalopathy / MGI
decreased vertical activity / MGI
abnormal inhibitory postsynaptic potential / MGI
abnormal inhibitory postsynaptic currents / MGI
no phenotypic analysis / MGI
increased neuron apoptosis / MGI
neuron degeneration / MGI
astrocytosis / MGI
abnormal voluntary movement / MGI
nervous system phenotype / MGI
abnormal nervous system morphology / MGI
impaired acrosome reaction / MGI
abnormal behavior / MGI
abnormal neuronal precursor proliferation / MGI
decreased susceptibility to prion infection / MGI
increased susceptibility to prion infection / MGI
behavior/neurological phenotype / MGI
immune system phenotype / MGI
teratozoospermia / MGI
brain vacuoles / MGI
abnormal brain white matter morphology / MGI
abnormal hippocampus CA1 region morphology / MGI
decreased neuron number / MGI
abnormal neuron differentiation / MGI
abnormal neuron proliferation / MGI
decreased brain copper level / MGI
enlarged brain ventricles / MGI
cerebellum atrophy / MGI
altered susceptibility to prion infection / 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 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

Example health report
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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-26Rchi/Cnrm