Overview

Also Known As:Fxn^null::MCK-Cre

Fxn^null::MCK-Cre mice harbor a frataxin global knock-out allele and a cardiac/skeletal muscle-specific Cre recombinase transgene. When heterozygous for Fxn^null and hemizygous or homozygous for MCK-Cre, these double mutant mice are "phenotypically normal" and are a parental control used to generate the progressive cardiomyopathy mouse line Fxn^flox/null::MCK-Cre (Stock No. 029720) - which is useful in studying Friedreich's Ataxia.

Donating Investigator

Cathleen Lutz, The Jackson Laboratory

Genetic overview

Genetic Background	Generation
	?+pN1F8 (2019-09-06 00:00:00)

Tg(Ckmm-cre)5Khn

Allele Type
Transgenic (Recombinase-expressing)

Fxn^em2.1Lutzy

Allele Type	Gene Symbol	Gene Name
Endonuclease-mediated (Null/Knockout)	Fxn	frataxin

View Genetics

Research Applications

Research Tools
Developmental Biology Research
Neurobiology Research
Cardiovascular Research

View All Research Applications

Base Price

Starting at:

$278.00 Domestic price for female 4-week

356.51 Domestic price for breeder pair

View Price List

Details

Detailed Description

Fxn^null::MCK-Cre mice harbor a frataxin global knock-out allele Fxn^null (Fxn^em2.1Lutzy) and the MCK-Cre transgene (Tg(Ckmm-cre)5Khn) that has Cre recombinase expression directed to cardiac and skeletal muscle. When heterozygous for the Fxn^null allele and hemizygous or homozygous for the MCK-Cre transgene, these double mutant mice are "phenotypically normal" and only used as a parental strain to generate the early-onset cardiomyopathy model of Friedreich's Ataxia, Fxn^flox/null::MCK-Cre (Stock No. 029720).

When singly heterozygous for the frataxin global null allele, mice are viable and fertile with normal lifespan. Mice homozygous for the frataxin global null allele are embryonic lethal - regardless of presence or absence of the MCK-Cre transgene.

When singly hemizygous or homozygous for the MCK-Cre transgene, mice are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities.

Development

These Fxn^null::MCK-Cre mice (Stock No. 029100) harbor a global null allele of mouse frataxin Fxn^null (Fxn^em2.1Lutzy; from Stock No. 028040) and the MCK-Cre transgene with the Ckmm promoter driving Cre recombinase expression (Tg(Ckmm-cre)5Khn; from Stock No. 006475). Each component is described below.

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 signal 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.

To generate the global knock-out allele Fxn^null (Fxn^em2.1Lutzy), male mice carrying the floxed exon 2 allele (from Stock No. 028520) were bred to female B6N.Cg-Tg(Sox2-cre)1Amc/J (Stock No. 014094). The resulting mice (exon 2 deleted from germline) were further bred to C57BL/6J to breed out the Cre recombinase transgene. Heterozygous intercrosses were performed to confirm the embryonic lethality of the null allele. Mice heterozygous for this frataxin global knock-out allele are available as Stock No. 028040. Fxn^null mice from Stock No. 028040 were used to generate Stock No. 029100.

The Tg(Ckmm-cre) transgene (MCK-Cre) was designed by Dr. C. Ronald Kahn (Joslin Diabetes Center) to have a Cre recombinase cDNA sequence (with a SV-40 large T antigen nuclear localization signal and polyA signal) inserted in place of the translation initiation site of the Ckm gene. C57BL/6-congenic MCK-Cre line 5 mice from Stock No. 006475 were used to generate Stock No. 029100.

Expression Data

Expressed Gene	cre, cre recombinase, bacteriophage P1
Site of Expression	skeletal and cardiac muscle
Site of Expression	Cardiac and skeletal muscle.

Control Suggestions

Approximate Controls

000664 C57BL/6J

Additional Information

Considerations for Choosing Controls

Genetics

Tg(Ckmm-cre)5Khn

Allele Symbol: Tg(Ckmm-cre)5Khn

Allele Name	transgene insertion 5, C Ronald Kahn
Allele Type	Transgenic (Recombinase-expressing)
Allele Synonym(s)	Ckm-cre; Ckmm-cre; Ckmm-NLS-cre; Cre^Mck; mckCRE; MCK-cre; MCKCre⁺; MCK-cre5; Tg(Ckm-cre)5Khn; Tg(Ckmm-cre)1Khn
Gene Symbol and Name	Tg(Ckmm-cre)5Khn, transgene insertion 5, C Ronald Kahn
Gene Synonym(s)
Promoter	Ckm, creatine kinase, muscle, mouse, laboratory
Expressed Gene	cre, cre recombinase, bacteriophage P1
Site of Expression	skeletal and cardiac muscle
Strain of Origin	FVB
Chromosome	UN
Molecular Note	A 6.5 kb genomic DNA fragment of the Ckmm gene containing the promoter and enhancer 1, untranslated exon 1, 3kb of intron 1 including the enhancer 2 region, and the first 16 bp of exon 2 drives expression of a modified cre with an SV40 large T antigen nuclear localization signal. Expression is directed to the heart and skeletal muscle.
Mutations Made By	C. Ronald Kahn, Joslin Diabetes Center

Fxn^em2.1Lutzy

Allele Symbol: Fxn^em2.1Lutzy

Allele Name	endonuclease-mediated mutation 2.1, Cathy Lutz
Allele Type	Endonuclease-mediated (Null/Knockout)
Allele Synonym(s)
Gene Symbol and Name	Fxn, frataxin
Gene Synonym(s)
Site of Expression	Cardiac and skeletal muscle.
Strain of Origin	C57BL/6J
Chromosome	19
Molecular Note	CRISPR/Cas9 genome editing was used to introduce loxP sites flanking exon 2. Cre-mediated recombination removed exon 2.

Disease/Phenotype

Disease Terms

Research Areas By Phenotype

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

Research Tools
- Cre-lox System
  - Cre Recombinase Expression
- Genetics Research
  - Mutagenesis and Transgenesis: Cre-lox System
- Cardiovascular Research
  - Cre-lox System
- Developmental Biology Research
  - Cre-lox System
Developmental Biology Research
- Embryonic Lethality (Homozygous)
Neurobiology Research
- Neuromuscular defects
- Ataxia (Movement) Defects
- Friedreich's Ataxia
- Cre-lox System
Cardiovascular Research
- Heart Abnormalities
  - cardiomyopathy

Mammalian Phenotype Terms by Genotype

References

Additional - Fxn^em2.1Lutzy related

Additional - Tg(Ckmm-cre)5Khn related

Technical Support

CONTACT TECHNICAL SUPPORT

Genotyping Protocols
- Standard PCR:Fxn-cKO
- Standard PCR:Fxn-KO
- Standard PCR:Tg(Ckmm-cre)5Khn
- Probe:Fxn-KO Probe Alternate1
- QPCR:Tg(Ckmm-cre)5Khn qPCR
- Genotyping resources and troubleshooting
Dietary Information
- New Diet as of March 2015: Lab Diet® 5K0Q (6% fat)
Breeding Considerations

Fxn^null::MCK-Cre mice are heterozygous for the frataxin global knockout allele Fxn^null (Fxn^em2.1Lutzy) on chromosome 19 and hemizygous or homozygous for the MCK-Cre transgene (Tg(Ckmm-cre)5Khn).

Mice homozygous for the frataxin global null allele are embryonic lethal - regardless of presence or absence of the MCK-Cre transgene. As such, we maintain our live colony by breeding mice heterozygous for Fxn^em2.1Lutzy and homozygous for Tg(Ckmm-cre)5Khn to mice wildtype at the Fxn locus and homozygous for Tg(Ckmm-cre)5Khn.

The expected coat color is black.
- Additional Breeding and Husbandry Support
Mating System
- Mice homozygous for the frataxin global null allele are embryonic lethal - regardless of presence or absence of the MCK-Cre transgene. As such, we maintain our live colony by breeding mice heterozygous for Fxn^em2.1Lutzy and homozygous for Tg(Ckmm-cre)5Khn to mice wildtype at the Fxn locus and homozygous for the transgene (i.e., C57BL/6J inbred mice). We also breed mice heterozygous for Fxn^em2.1Lutzy and homozygous for the transgene to mice wildtype at the Fxn locus and homozygous for Tg(Ckmm-cre)5Khn
Citation
When using the Fxn^null::MCK-Cre mouse strain in a publication, please cite the originating article(s) and include JAX stock #029100 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

Breeder Pair

Sex

Genotype

Price

Female
Male

Cryorecovery - Pricing

Service/Product	Description	Price
	Heterozygous or Wildtype forÊFxn<em#Lutzy>and Hemizygous or Non carrier forÊTg(Ckmm-cre)5Khn

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:Fxnnull::MCK-Cre

Donating Investigator

Genetic overview

Research Applications

Base Price

Detailed Description

Development

Expression Data

Control Suggestions

Approximate Controls

Additional Information

Tg(Ckmm-cre)5Khn

Allele Symbol: Tg(Ckmm-cre)5Khn

Fxnem2.1Lutzy

Allele Symbol: Fxnem2.1Lutzy

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 - Fxnem2.1Lutzy related

Additional - Tg(Ckmm-cre)5Khn related

Genotyping Protocols

Dietary Information

Breeding Considerations

Mating System

Citation

Animal Health Reports

Live Mouse

Breeder Pair

Cryorecovery - Pricing

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^null::MCK-Cre

Fxn^em2.1Lutzy

Allele Symbol: Fxn^em2.1Lutzy

Additional - Fxn^em2.1Lutzy related