B6J.Cg-Fus^{tm3.1(FUS)Emcf}/EmcfH

Status	Available to order
EMMA ID	EM:13073
Citation information	RRID:IMSR_EM:13073 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	B6J.Cg-Fus^{tm3.1(FUS)Emcf}/EmcfH
Alternative name	C57BL/6J-Fus tm1.1(FUS)Emcf/H
Strain type	Targeted Mutant Strains : Knock-in
Allele/Transgene symbol	Fus^{tm3.1(FUS)Emcf}
Gene/Transgene symbol	Fus

Information from provider

Provider	Elizabeth Fisher
Provider affiliation	Mammalian Genetics Unit, MRC Harwell Institute
Genetic information	Mice carry the human fused in sarcoma (FUS) gene at the endogenous locus. The Fus gene has been humanised (human orthologous sequence replacing mouse sequence), starting from the ATG start codon, including introns and 3' UTR.
Phenotypic information	Homozygous: Homozygous mice are viable, fertile and produced at normal mendelian ratios. Survival is normal up to 18 months. No obvious motor, or other, phenotype. Heterozygous: Heterozygous mice are viable, fertile, and produced at normal mendelian ratios. Survival is normal up to 18 months. No obvious motor, or other, phenotype.
Breeding history	Targeting was performed in 129 R1 ES cells. Following germ-line transmission in mice, were modified and used for implantation. The line has now been backcrossed 10 times onto C57BL/6J so is considered congenic.
References	Generation and analysis of innovative genomically humanized knockin SOD1, TARDBP (TDP-43), and FUS mouse models.;Devoy Anny, Price Georgia, De Giorgio Francesca, Bunton-Stasyshyn Rosie, Thompson David, Gasco Samanta, Allan Alasdair, Codner Gemma F, Nair Remya R, Tibbit Charlotte, McLeod Ross, Ali Zeinab, Noda Judith, Marrero-Gagliardi Alessandro, Brito-Armas José M, Williams Chloe, Öztürk Muhammet M, Simon Michelle, O'Neill Edward, Bryce-Smith Sam, Harrison Jackie, Atkins Gemma, Corrochano Silvia, Stewart Michelle, Gilthorpe Jonathan D, Teboul Lydia, Acevedo-Arozena Abraham, Fisher Elizabeth M C, Cunningham Thomas J, ;2021;iScience;24;103463; 34988393 Sectioning and Counting of Motor Neurons in the L3 to L6 Region of the Adult Mouse Spinal Cord.;Austin Adele, Beresford Lynn, Price Georgia, Cunningham Tom, Kalmar Bernadett, Yon Marianne, ;2022;Current protocols;2;e428; 35617451
Homozygous fertile	yes
Homozygous viable	yes
Homozygous matings required	no
Immunocompromised	no

Information from EMMA

Archiving centre	Mary Lyon Centre at MRC Harwell, Oxford, United Kingdom

Disease and phenotype information

Orphanet associated rare diseases, based on orthologous gene matching

Amyotrophic lateral sclerosis / Orphanet_803
Juvenile amyotrophic lateral sclerosis / Orphanet_300605

IMPC phenotypes (gene matching)

increased respiratory quotient / IMPC
increased prepulse inhibition / IMPC
preweaning lethality, complete penetrance / IMPC
hypoplasia / IMPC
abnormal embryo size / IMPC

MGI phenotypes (gene matching)

small thymus / MGI
motor neuron degeneration / MGI
decreased motor neuron number / MGI
abnormal neuromuscular synapse morphology / MGI
small testis / MGI
decreased body weight / MGI
decreased body size / MGI
abnormal gait / MGI
abnormal suckling behavior / MGI
impaired limb coordination / MGI
increased mortality induced by gamma-irradiation / MGI
postnatal growth retardation / MGI
reduced female fertility / MGI
male infertility / MGI
decreased litter size / MGI
premature death / MGI
no abnormal phenotype detected / MGI
lymphoid hypoplasia / MGI
abnormal immunoglobulin level / MGI
no phenotypic analysis / MGI
abnormal chromosome morphology / MGI
aneuploidy / MGI
chromosome breakage / MGI
decreased lymphocyte cell number / MGI
decreased B cell number / MGI
abnormal male meiosis / MGI
abnormal hippocampus pyramidal cell morphology / MGI
postnatal lethality, incomplete penetrance / MGI
neonatal lethality, complete penetrance / MGI

Literature references

Generation and analysis of innovative genomically humanized knockin SOD1, TARDBP (TDP-43), and FUS mouse models.;Devoy Anny, Price Georgia, De Giorgio Francesca, Bunton-Stasyshyn Rosie, Thompson David, Gasco Samanta, Allan Alasdair, Codner Gemma F, Nair Remya R, Tibbit Charlotte, McLeod Ross, Ali Zeinab, Noda Judith, Marrero-Gagliardi Alessandro, Brito-Armas José M, Williams Chloe, Öztürk Muhammet M, Simon Michelle, O'Neill Edward, Bryce-Smith Sam, Harrison Jackie, Atkins Gemma, Corrochano Silvia, Stewart Michelle, Gilthorpe Jonathan D, Teboul Lydia, Acevedo-Arozena Abraham, Fisher Elizabeth M C, Cunningham Thomas J, ;2021;iScience;24;103463; 34988393
Sectioning and Counting of Motor Neurons in the L3 to L6 Region of the Adult Mouse Spinal Cord.;Austin Adele, Beresford Lynn, Price Georgia, Cunningham Tom, Kalmar Bernadett, Yon Marianne, ;2022;Current protocols;2;e428; 35617451

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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^*
Tissue - Types of tissue, service fee and delivery time available upon request

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B6J.Cg-Fustm3.1(FUS)Emcf/EmcfH