JAX Mouse Strain Datasheet - 012253

B6.Cg-Fxn^tm1Mkn Tg(FXN)YG8Pook/J

Stock No:: 012253

Cryo Recovery

Overview

Also Known As: YG8R

This human FXN YAC transgenic mouse model has the mouse frataxin knockout allele (Fxn^-) and the human FXN YAC transgene from founder YG8 (carrying two tandem copies of the human FXN gene with ~82 and ~190 GAA trinucleotide sequence repeats). Mice homozygous for the knockout and hemizygous for the YG8 transgene, called YG8R mice, are rescued from knockout lethality and have transgene expression that models the phenotype of Friedreich's Ataxia (FRDA).

Donating Investigator

Mark A Pook, Brunel University

Genetic overview

Genetic Background	Generation

Fxn^tm1Mkn

Allele Type	Gene Symbol	Gene Name
Targeted (Null/Knockout)	Fxn	frataxin

Tg(FXN)YG8Pook

Allele Type
Transgenic (Humanized sequence, Inserted expressed sequence)

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Research Applications

Neurobiology Research

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Base Price

Starting at:

$2,595.00 Domestic price Cryo Recovery

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Details

Important Note

This strain is maintained heterozygous for the targeted mutation and hemizygous for the transgene.

Detailed Description

Importation of this model was supported by the Friedreich's Ataxia Research Alliance (FARA).

The YG8 transgenic founder line carries two tandem copies of the human FXN gene with ~82 and ~190 GAA trinucleotide sequence repeats. Mice that are heterozygous for the Fxn^- knockout allele and hemizygous for the YG8 transgene are viable and fertile. High levels of human FXN gene product (mRNA or protein) are detected by RT-PCR and Western blot analysis. Approximately 40-50% of the endogenous mouse Fxn gene product (protein) is detected by Western blot analysis in mice heterozygous for the Fxn^- knockout allele alone. Mice homozygous for the Fxn^- knockout allele and hemizygous for the YG8 transgene, called YG8R mice, are rescued from knockout lethality and have transgene expression that results in an age-dependent, tissue-specific expansion of the GAA repeat, with expansion accumulation observed in the CNS (particularly cerebellum), similar to the human pathology of Friedreich's Ataxia. The GAA triplet repeats exhibit intergenerational instability.

In an attempt to offer alleles on well-characterized or multiple genetic backgrounds, alleles are frequently moved to a genetic background different from that on which an allele was first characterized. This is the case for the strain above. It should be noted that the phenotype could vary from that originally described. We will modify the strain description if necessary as published results become available.

It should be noted that the YAC transgenic FRDA mouse models available from The Jackson Laboratory (YG22R, YG8R and YG8sR) may have different GAA repeat sizes than the animals used in the publications described below.
Publicationsfrom Dr. Mark A. Pook (Brunel University London) in 2014-2015 examine the correlation between the FRDA-like pathological phenotype and frataxin-deficiency in the YAC transgenic FRDA mouse models YG22R (two copies of the FRDA transgene; 190 GAA repeats expanded to ~170-260), YG8R (two copies of the FRDA transgene; 90-190 GAA repeats expanded to ~120-220) and YG8sR (a contraction of the transgene to a single copy; ~120-150 GAA repeats) compared to Y47R control mice (one copy of the FRDA transgene with a stable 9 GAA repeat) and C57BL6/J wildtype mice. The results described below are all in comparison to control mice.
The three YAC transgenic FRDA mouse models (YG8R, YG22R and YG8sR) had a progressive decrease in the motor coordination; the degree of impairment was most significant in YG8R mice. All three models exhibited GAA repeat somatic instability in the brain, cerebellum and liver, as well as exhibited glucose intolerance and insulin hypersensitivity. The greatest FXN deficiency of the three models tested was in YG8sR. Expression levels of the transgenes in various tissues are reported in the Pook publications.

Development

These double mutant mice, heterozygous for the targeted mutation and hemizygous for the transgene, were generated by crossing transgenic mice (founder line YG8) with Fxn^tm1Mkn targeted mutant mice.

Drs. Michel Koenig and Helene Puccio generated the Fxn targeted mutation allele by disrupting exon 4 and flanking sequences with a loxP site flanked PGK-neo cassette. The construct was electroporated into 129/Sv derived embryonic stem (ES) cells. Correctly targeted ES cells were injected into C57BL/6J blastocysts. The resulting chimeric animals were crossed to C57BL/6J mice, and then backcrossed to C57BL/6J for five generations.

The transgenic construct consisting of a 370kb YAC insert that includes the entire human FXN gene with GAA triplet repeat sequences, was injected into fertilized CBA X C57BL/6 hybrid fertilized oocytes. Founder line YG8 was established, carrying 2 tandem copies of the human FXN gene with GAA trinucleotide sequence repeats as low as 122 and as high as 207. Founder animals were bred to wildtype C57BL/6J mice for five generations.

The two lines were then crossed to generate the double mutant. The Donating Investigator breeds mice that are heterozygous for the Fxn^tm1Mkn targeted mutation and hemizygous for the YG8 transgene to mice that are heterozygous for the Fxn^tm1Mkn targeted mutation to obtain mice homozygous for the Fxn^tm1Mkn targeted mutation and hemizygous for the YG8 transgene.

SNP (single nucleotide polymorphism) analysis performed by The Jackson Laboratory revealed that the mice originally imported were on a mixed genetic background. The mixed genetic background mice were then backcrossed to C57BL/6J for five generations.

Expression Data

Expressed Gene	Fxn, frataxin, mouse, laboratory
Site of Expression
Expressed Gene	FXN, frataxin, human
Site of Expression

Control Suggestions

000664 C57BL/6J

Additional Information

Considerations for Choosing Controls

Selected References

Al-Mahdawi S; Pinto RM; Varshney D; Lawrence L; Lowrie MB; Hughes S; Webster Z; Blake J; Cooper JM; King R; Pook MA. 2006. GAA repeat expansion mutation mouse models of Friedreich ataxia exhibit oxidative stress leading to progressive neuronal and cardiac pathology. Genomics 88(5):580-90. PubMed: 16919418 MGI: J:114840
Anjomani Virmouni S; Sandi C; Al-Mahdawi S; Pook MA. 2014. Cellular, molecular and functional characterisation of YAC transgenic mouse models of Friedreich ataxia. PLoS One 9(9):e107416. PubMed: 25198290 MGI: J:217861

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Genetics

Fxn^tm1Mkn

Allele Symbol: Fxn^tm1Mkn

Allele Name	targeted mutation 1, Michel Koenig
Allele Type	Targeted (Null/Knockout)
Allele Synonym(s)	Frda^-; Frda^del4
Gene Symbol and Name	Fxn, frataxin
Gene Synonym(s)	CyaY; FA; FARR; FRDA; Frda; Frda; Friedreich ataxia; RGD1565754; X25
Expressed Gene	Fxn, frataxin, mouse, laboratory
Strain of Origin	129/Sv
Chromosome	19
Molecular Note	A loxP-flanked PGK-neomycin resistance cassette replaced a genomic DNA fragment containing exon 4, which is highly conserved and often mutated in humans. An additional line was also produced in which the loxP flanked neomycin cassette was removed by Cre mediated recombination, but no distinction was made between these alleles in the original reference. From J:90098: The presence of a human FRDA transgene in hemizygous form in a Frda^tm1Mkn homozygous null background rescues the embryonic lethal phenotype and complements for the loss of endogenous mouse frataxin.

Tg(FXN)YG8Pook

Allele Symbol: Tg(FXN)YG8Pook

Allele Name	transgene insertion YG8, Mark A Pook
Allele Type	Transgenic (Humanized sequence, Inserted expressed sequence)
Allele Synonym(s)	YG8
Gene Symbol and Name	Tg(FXN)YG8Pook, transgene insertion YG8, Mark A Pook
Gene Synonym(s)	YG8
Promoter	FXN, frataxin, human
Expressed Gene	FXN, frataxin, human
Strain of Origin	CBA x C57BL/6
Chromosome	UN
General Note	Line YG22 exhibits slightly greater age-related repeat instability, with a bias toward repeat expansion.
Molecular Note	The transgenic construct consists of a 370kb YAC insert that includes the entire human FXN gene with GAA triplet repeat sequences, and was injected into fertilized CBA X C57BL/6 hybrid fertilized oocytes. Founder line YG8 was established, carrying 2 tandem copies of the human FXN gene with two GAA trinucleotide repeat sequences of 82 and 190 repeats. This line displayed a transmission rate of 52% to offspring.

Disease/Phenotype

Disease Terms

Research Areas By Genotype

This mouse can be used to support research in many areas including:

Neurobiology Research
- Friedreich's Ataxia

Mammalian Phenotype Terms by Genotype

The following phenotype information is associated with a similar, but not exact match to this JAX^® Mice strain

Genotype: Fxn^tm1Mkn/Fxn^tm1Mkn Tg(FXN)YG8Pook/0
involves: 129/Sv * C57BL/6 * CBA

mortality/aging

mortality/aging
- embryonic lethality seen for Fxn-deficient embryos is recued by transgene expression; normal numbers of offspring are recovered and mice show normal lifespans, surviving to at least 2 years of age

growth/size/body region phenotype

increased body weight
- significant increase is observed from 9 months of age

nervous system phenotype

abnormal action potential
- slight decrease in sensory action potential in 20-months old mice is detected, suggesting a mild progressive sensory neuropathy

abnormal axon morphology
- at 20 months, some axons in lumbar DRG display swelling and secondary demyelination

abnormal dorsal root ganglion morphology
- 16% of cervical DRG sections display vacuoles
- in mice >1 year of age, vacuoles are observed within the DRG in the cervical region

abnormal lumbar dorsal root ganglion morphology
- 70% of lumbar DRG sections examined have vacuoles
- prominent, giant vacuoles (round, singular or multiple) are observed in large sensory neuronal bodies of DRG in mice between 6 and 12 months, but not in controls; peripheral margination of nucleus in many large neuronal cell bodies with or without vacuoless

brain inflammation
- cerebellum shows increased induction of inflammation in response to lipopolysaccharide (LPS) treatment

chromatolysis
- some regions of lumbar DRG are devoid of cytoplasmic organelles at 20 months

decreased nerve conduction velocity
- slight decrease in sensory nerve conduction velocity in 20-months old mice is detected, suggesting a mild progressive sensory neuropathy

nervous system phenotype
- no neuronal histopathology is detected in cerebellar Purkinje cells or granule cells, and no abnormalities in brain or spinal cord regions are seen

neuron degeneration
- degenerating large DRG neurons are detected, with evidence of oxidative stress and mitochondrial dysfunction

behavior/neurological phenotype

hypoactivity
- mice show a significant decrease in locomotor activity in the open field from 6 months of age

impaired coordination
- reduced rotarod performance is exhibited by mice from 3 months of age

cardiovascular system phenotype

abnormal myocardial fiber morphology
- patches of lipofuscin deposition and lysosymes, with accumulation of dispersed free glycogen disrupt regular arrangement of mitochondria within cardiomyocytes at 20 months

cellular phenotype

abnormal respiratory electron transport chain
- mitochondrial respiratory chain function is decreased compared to controls

oxidative stress
- in 6-9 month old animals, levels of oxidized proteins are increased, most significantly in cerebrum and cerebellum, with other significant increases seen in heart and skeletal muscle
- increased lipid peroxidation is detected in heart samples

muscle phenotype

abnormal myocardial fiber morphology
- patches of lipofuscin deposition and lysosymes, with accumulation of dispersed free glycogen disrupt regular arrangement of mitochondria within cardiomyocytes at 20 months

homeostasis/metabolism phenotype

abnormal enzyme/coenzyme activity
- aconitase activity is decreased to <70% of wild-type levels

immune system phenotype

brain inflammation
- cerebellum shows increased induction of inflammation in response to lipopolysaccharide (LPS) treatment

References

Al-Mahdawi S; Pinto RM; Varshney D; Lawrence L; Lowrie MB; Hughes S; Webster Z; Blake J; Cooper JM; King R; Pook MA. 2006. GAA repeat expansion mutation mouse models of Friedreich ataxia exhibit oxidative stress leading to progressive neuronal and cardiac pathology. Genomics 88(5):580-90. PubMed: 16919418 MGI: J:114840
Anjomani Virmouni S; Sandi C; Al-Mahdawi S; Pook MA. 2014. Cellular, molecular and functional characterisation of YAC transgenic mouse models of Friedreich ataxia. PLoS One 9(9):e107416. PubMed: 25198290 MGI: J:217861

Additional - Fxn^tm1Mkn related

Al-Mahdawi S; Pinto RM; Ruddle P; Carroll C; Webster Z; Pook M. 2004. GAA repeat instability in Friedreich ataxia YAC transgenic mice. Genomics 84(2):301-10. PubMed: 15233994 MGI: J:91581
Anjomani Virmouni S; Ezzatizadeh V; Sandi C; Sandi M; Al-Mahdawi S; Chutake Y; Pook MA. 2015. A novel GAA repeat expansion-based mouse model of Friedreich ataxia. Dis Model Mech :. PubMed: 25681319 MGI: J:217862
Cloonan SM; Glass K; Laucho-Contreras ME; Bhashyam AR; Cervo M; Pabon MA; Konrad C; Polverino F; Siempos II; Perez E; Mizumura K; Ghosh MC; Parameswaran H; Williams NC; Rooney KT; Chen ZH; Goldklang MP; Yuan GC; Moore SC; Demeo DL; Rouault TA; D'Armiento JM; Schon EA; Manfredi G; Quackenbush J; Mahmood A; Silverman EK; Owen CA; Choi AM. 2016. Mitochondrial iron chelation ameliorates cigarette smoke-induced bronchitis and emphysema in mice. Nat Med 22(2):163-74. PubMed: 26752519 MGI: J:233150
Cossee M; Puccio H; Gansmuller A; Koutnikova H; Dierich A; LeMeur M; Fischbeck K; Dolle P; Koenig M. 2000. Inactivation of the Friedreich ataxia mouse gene leads to early embryonic lethality without iron accumulation. Hum Mol Genet 9(8):1219-26. PubMed: 10767347 MGI: J:62185
Hayashi G; Shen Y; Pedersen TL; Newman JW; Pook M; Cortopassi G. 2014. Frataxin deficiency increases cyclooxygenase 2 and prostaglandins in cell and animal models of Friedreich's ataxia. Hum Mol Genet 23(25):6838-47. PubMed: 25104852 MGI: J:216422
Jasoliya MJ; McMackin MZ; Henderson CK; Perlman SL; Cortopassi GA. 2017. Frataxin deficiency impairs mitochondrial biogenesis in cells, mice and humans. Hum Mol Genet 26(14):2627-2633. PubMed: 28444186 MGI: J:242978
Jones J; Estirado A; Redondo C; Martinez S. 2013. Stem cells from wildtype and friedreich's ataxia mice present similar neuroprotective properties in dorsal root ganglia cells. PLoS One 8(5):e62807. PubMed: 23671637 MGI: J:202178
Miranda CJ; Santos MM; Ohshima K; Smith J; Li L; Bunting M; Cossee M; Koenig M; Sequeiros J; Kaplan J; Pandolfo M. 2002. Frataxin knockin mouse. FEBS Lett 512(1-3):291-7. PubMed: 11852098 MGI: J:109164
Molla B; Riveiro F; Bolinches-Amoros A; Munoz-Lasso DC; Palau F; Gonzalez-Cabo P. 2016. Two different pathogenic mechanisms, dying-back axonal neuropathy and pancreatic senescence, are present in the YG8R mouse model of Friedreich ataxia. Dis Model Mech :. PubMed: 27079523 MGI: J:232478
Pook MA; Al-Mahdawi S; Carroll CJ; Cossee M; Puccio H; Lawrence L; Clark P; Lowrie MB; Bradley JL; Cooper JM; Koenig M; Chamberlain S. 2001. Rescue of the Friedreich's ataxia knockout mouse by human YAC transgenesis. Neurogenetics 3(4):185-93. PubMed: 11714098 MGI: J:213628
Sahdeo S; Scott BD; McMackin MZ; Jasoliya M; Brown B; Wulff H; Perlman SL; Pook MA; Cortopassi GA. 2014. Dyclonine rescues frataxin deficiency in animal models and buccal cells of patients with Friedreich's ataxia. Hum Mol Genet 23(25):6848-62. PubMed: 25113747 MGI: J:216419
Sandi C; Pinto RM; Al-Mahdawi S; Ezzatizadeh V; Barnes G; Jones S; Rusche JR; Gottesfeld JM; Pook MA. 2011. Prolonged treatment with pimelic o-aminobenzamide HDAC inhibitors ameliorates the disease phenotype of a Friedreich ataxia mouse model. Neurobiol Dis 42(3):496-505. PubMed: 21397024 MGI: J:172875
Santos MM; Miranda CJ; Levy JE; Montross LK; Cossee M; Sequeiros J; Andrews N; Koenig M; Pandolfo M. 2003. Iron metabolism in mice with partial frataxin deficiency. Cerebellum 2(2):146-53. PubMed: 12880182 MGI: J:112348
Sarsero JP; Holloway TP; Li L; Finkelstein DI; Ioannou PA. 2014. Rescue of the Friedreich ataxia knockout mutation in transgenic mice containing an FXN-EGFP genomic reporter. PLoS One 9(3):e93307. PubMed: 24667739 MGI: J:214147
Sarsero JP; Li L; Holloway TP; Voullaire L; Gazeas S; Fowler KJ; Kirby DM; Thorburn DR; Galle A; Cheema S; Koenig M; Williamson R; Ioannou PA. 2004. Human BAC-mediated rescue of the Friedreich ataxia knockout mutation in transgenic mice. Mamm Genome 15(5):370-82. PubMed: 15170226 MGI: J:90098
Tomassini B; Arcuri G; Fortuni S; Sandi C; Ezzatizadeh V; Casali C; Condo I; Malisan F; Al-Mahdawi S; Pook M; Testi R. 2012. Interferon gamma upregulates frataxin and corrects the functional deficits in a Friedreich ataxia model. Hum Mol Genet 21(13):2855-61. PubMed: 22447512 MGI: J:184617

Additional - Tg(FXN)YG8Pook related

Al-Mahdawi S; Pinto RM; Ruddle P; Carroll C; Webster Z; Pook M. 2004. GAA repeat instability in Friedreich ataxia YAC transgenic mice. Genomics 84(2):301-10. PubMed: 15233994 MGI: J:91581
Anjomani Virmouni S; Ezzatizadeh V; Sandi C; Sandi M; Al-Mahdawi S; Chutake Y; Pook MA. 2015. A novel GAA repeat expansion-based mouse model of Friedreich ataxia. Dis Model Mech :. PubMed: 25681319 MGI: J:217862
Bourn RL; De Biase I; Pinto RM; Sandi C; Al-Mahdawi S; Pook MA; Bidichandani SI. 2012. Pms2 suppresses large expansions of the (GAA.TTC)n sequence in neuronal tissues. PLoS One 7(10):e47085. PubMed: 23071719 MGI: J:192085
Ezzatizadeh V; Pinto RM; Sandi C; Sandi M; Al-Mahdawi S; Te Riele H; Pook MA. 2012. The mismatch repair system protects against intergenerational GAA repeat instability in a Friedreich ataxia mouse model. Neurobiol Dis 46(1):165-71. PubMed: 22289650 MGI: J:182296
Hayashi G; Shen Y; Pedersen TL; Newman JW; Pook M; Cortopassi G. 2014. Frataxin deficiency increases cyclooxygenase 2 and prostaglandins in cell and animal models of Friedreich's ataxia. Hum Mol Genet 23(25):6838-47. PubMed: 25104852 MGI: J:216422
Jones J; Estirado A; Redondo C; Martinez S. 2013. Stem cells from wildtype and friedreich's ataxia mice present similar neuroprotective properties in dorsal root ganglia cells. PLoS One 8(5):e62807. PubMed: 23671637 MGI: J:202178
Jones J; Estirado A; Redondo C; Pacheco-Torres J; Sirerol-Piquer MS; Garcia-Verdugo JM; Martinez S. 2015. Mesenchymal stem cells improve motor functions and decrease neurodegeneration in ataxic mice. Mol Ther 23(1):130-8. PubMed: 25070719 MGI: J:232156
Molla B; Riveiro F; Bolinches-Amoros A; Munoz-Lasso DC; Palau F; Gonzalez-Cabo P. 2016. Two different pathogenic mechanisms, dying-back axonal neuropathy and pancreatic senescence, are present in the YG8R mouse model of Friedreich ataxia. Dis Model Mech :. PubMed: 27079523 MGI: J:232478
Sahdeo S; Scott BD; McMackin MZ; Jasoliya M; Brown B; Wulff H; Perlman SL; Pook MA; Cortopassi GA. 2014. Dyclonine rescues frataxin deficiency in animal models and buccal cells of patients with Friedreich's ataxia. Hum Mol Genet 23(25):6848-62. PubMed: 25113747 MGI: J:216419
Sandi C; Pinto RM; Al-Mahdawi S; Ezzatizadeh V; Barnes G; Jones S; Rusche JR; Gottesfeld JM; Pook MA. 2011. Prolonged treatment with pimelic o-aminobenzamide HDAC inhibitors ameliorates the disease phenotype of a Friedreich ataxia mouse model. Neurobiol Dis 42(3):496-505. PubMed: 21397024 MGI: J:172875
Tomassini B; Arcuri G; Fortuni S; Sandi C; Ezzatizadeh V; Casali C; Condo I; Malisan F; Al-Mahdawi S; Pook M; Testi R. 2012. Interferon gamma upregulates frataxin and corrects the functional deficits in a Friedreich ataxia model. Hum Mol Genet 21(13):2855-61. PubMed: 22447512 MGI: J:184617

Service	Genotype	Price
	Heterozygous or wild-type for Fxn<tm1Mkn> , Hemizygous or Non Carrier for Tg(FXN)YG8Pook

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Also Known As: YG8R

Donating Investigator

Genetic overview

Research Applications

Base Price

Important Note

Detailed Description

Development

Expression Data

Control Suggestions

Additional Information

Selected References

Fxntm1Mkn

Allele Symbol: Fxntm1Mkn

Tg(FXN)YG8Pook

Allele Symbol: Tg(FXN)YG8Pook

Disease Terms

Research Areas By Genotype

This mouse can be used to support research in many areas including:

Mammalian Phenotype Terms by Genotype

Genotype: Fxntm1Mkn/Fxntm1Mkn Tg(FXN)YG8Pook/0involves: 129/Sv * C57BL/6 * CBA

mortality/aging

growth/size/body region phenotype

nervous system phenotype

behavior/neurological phenotype

cardiovascular system phenotype

cellular phenotype

muscle phenotype

homeostasis/metabolism phenotype

immune system phenotype

References

Additional - Fxntm1Mkn related

Additional - Tg(FXN)YG8Pook related

Genotyping Protocols

Breeding Considerations

Citation

Animal Health Reports

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200

Live Mouse

Cryorecovery - Pricing

Progeny testing is not required

Payment Terms and Conditions

The Jackson Laboratory's Genotype Promise

Terms of Use

Additional Use Restrictions Apply

Licensing Information

JAX® Mice, Products & Services Conditions of Use

No Warranty

Credit for PRODUCTS or SERVICES

Animal Care and Use for SERVICES

No Liability

Fxn^tm1Mkn

Allele Symbol: Fxn^tm1Mkn

Genotype: Fxn^tm1Mkn/Fxn^tm1Mkn Tg(FXN)YG8Pook/0
involves: 129/Sv * C57BL/6 * CBA

Additional - Fxn^tm1Mkn related