Overview

Also Known As:Fxn^flox (floxed exon 2)

Fxn^flox (floxed exon 2) mice have a CRISPR/Cas9-generated, Cre-conditional frataxin allele - and may be useful in studying Friedreich's Ataxia. In addition, these mice are a parental control used to generate the progressive cardiomyopathy mouse line Fxn^flox/null::MCK-Cre (Stock No. 029720) and the Friedreich's Ataxia mouse model, Fxn^flox/null::PV-Cre (Stock No. 029721).

Our preclinical efficacy testing services offer scientific expertise and an array of target-based and phenotype-based outcome measures, both in vivo and at endpoint, for flexible study designs and assay development in mouse models of Friedreich's Ataxia. See our full service platform.

Donating Investigator

Cathleen Lutz, The Jackson Laboratory

Genetic overview

Genetic Background	Generation
	N3F2pF1

Fxn^em2Lutzy

Allele Type	Gene Symbol	Gene Name
Endonuclease-mediated (Conditional ready (e.g. floxed), No functional change)	Fxn	frataxin

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Research Applications

Neurobiology Research
Research Tools
Cardiovascular Research

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Base Price

Starting at:

$255.00 Domestic price for female 4-week

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Details

Detailed Description

The mitochondrial frataxin protein (encoded by Fxn) is involved in mitochondrial iron transport and respiration regulation. FXN mutations are associated with Friedreich's Ataxia.

The frataxin floxed exon 2 allele (Fxn^flox) has loxP sites flanking exon 2. Mice homozygous for this floxed allele are viable and fertile with no reported gross phenotypic or behavioral abnormalities.

Upon exposure to Cre recombinase, the floxed sequences are deleted - resulting in a null allele. For example, breeding to germline Cre-expressing mice (e.g., Sox2-Cre; Stock No. 014094) generates mice with the frataxin global knockout allele (Fxn^null; Stock No. 028040). Mice homozygous for the global knockout allele (Fxn^null/null) exhibit embryonic lethality.

Development

The frataxin floxed exon 2 allele (Fxn^flox ; Fxn^em2Lutzy) was made by Dr. Cat Lutz at The Jackson Laboratory using CRISPR/cas9 endonuclease-mediated genome editing. Plasmids encoding a single guide RNA designed to introduce loxP sites flanking exon 2 in the Fxn gene and the CRISPR/cas9 nuclease were introduced into the cytoplasm C57BL/6J-derived fertilized eggs with well recognized pronuclei. Correctly targeted embryos were transferred to pseudopregnant females. Correctly targeted pups were identified by sequencing and PCR. Mice from founder 466 were further bred to C57BL/6J inbred mice (Stock No. 000664) to develop the frataxin floxed exon 2 colony as Stock No. 028520.

Control Suggestions

Wild-type from the colony

Approximate Controls

000664 C57BL/6J

Additional Information

Considerations for Choosing Controls

Genetics

Fxn^em2Lutzy

Allele Symbol: Fxn^em2Lutzy

Allele Name	endonuclease-mediated mutation 2, Cathy Lutz
Allele Type	Endonuclease-mediated (Conditional ready (e.g. floxed), No functional change)
Allele Synonym(s)
Gene Symbol and Name	Fxn, frataxin
Gene Synonym(s)
Strain of Origin	C57BL/6J
Chromosome	19
Molecular Note	CRISPR/Cas9 genome editing is used to introduce loxP sites flanking exon 2.

Disease/Phenotype

Disease Terms

Research Areas By Phenotype

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

Neurobiology Research
- Cre-lox System
  - loxP-flanked Sequences
- Friedreich's Ataxia
- Ataxia (Movement) Defects
- Neuromuscular defects
Research Tools
- Cardiovascular Research
  - Cre-lox System
- Genetics Research
  - Mutagenesis and Transgenesis: Cre-lox System
- Developmental Biology Research
  - Cre-lox System
- Cre-lox System
  - loxP-flanked Sequences
Cardiovascular Research
- Heart Abnormalities
  - cardiomyopathy

Mammalian Phenotype Terms by Genotype

References

Additional - Fxn^em2Lutzy related

Technical Support

CONTACT TECHNICAL SUPPORT

Genotyping Protocols
- Standard PCR:Fxn-cKO
- Genotyping resources and troubleshooting
Breeding Considerations

Mice homozygous for the floxed allele are viable and fertile with no reported gross phenotypic or behavioral abnormalities. When maintaining a live colony, heterozygous mice may be bred together, to wildtype mice from the colony or to C57BL/6J inbred mice (Stock No. 000664). Alternatively, homozygous mice may be bred together.
- Additional Breeding and Husbandry Support
Citation
When using the Fxn^flox (floxed exon 2) mouse strain in a publication, please cite the originating article(s) and include JAX stock #028520 in your Materials and Methods section.

Animal Health Reports

Facility Barrier Level Descriptions

AX18 (Maximum)

Pricing & Availability

Available

Domestic
International

Live Mouse

Age

Genotype

Price

weeks

Cryorecovery - Pricing

Service/Product	Description	Price
	Heterozygous or wildtype for Fxn<em2Lutzy>

Related Products and Services

Frozen Mouse Embryo	C57BL/6J-Fxn<em2Lutzy>/J Frozen Embryo	$2595.00
Frozen Mouse Embryo	C57BL/6J-Fxn<em2Lutzy>/J Frozen Embryo	$2595.00
Frozen Mouse Embryo	C57BL/6J-Fxn<em2Lutzy>/J Frozen Embryo	$3373.50
Frozen Mouse Embryo	C57BL/6J-Fxn<em2Lutzy>/J Frozen Embryo	$3373.50

Payment Terms and Conditions

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The Jackson Laboratory's Genotype Promise

The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project. We do not guarantee breeding performance and therefore suggest that investigators order more than one breeding pair to avoid delays in their research.

Terms Of Use

General Terms and Conditions

Questions about Terms of Use

Additional Use Restrictions Apply

The use of this mouse model is subject to the terms and conditions of the Limited License from The Broad Institute.

The use of this mouse model is subject to the terms and conditions of the Limited Use Label License from Caribou Biosciences, Inc.

Licensing Information

Phone: 207-288-6470

Email: TechTran@jax.org

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Also Known As:Fxnflox (floxed exon 2)

Donating Investigator

Genetic overview

Research Applications

Base Price

Detailed Description

Development

Control Suggestions

Approximate Controls

Additional Information

Fxnem2Lutzy

Allele Symbol: Fxnem2Lutzy

Disease Terms

Research Areas By Phenotype

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

Mammalian Phenotype Terms by Genotype

References

Additional - Fxnem2Lutzy related

Genotyping Protocols

Breeding Considerations

Citation

Animal Health Reports

Live Mouse

Cryorecovery - Pricing

Related Products and Services

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

Also Known As:Fxn^flox (floxed exon 2)

Fxn^em2Lutzy

Allele Symbol: Fxn^em2Lutzy

Additional - Fxn^em2Lutzy related