Overview

Also Known As:FVB;B6.Tg(FXN);Fxn-

These FVB;B6.Tg(FXN);Fxn- mice harbor the the FXN*500GAA transgene (Tg(FXN)1Sars) and frataxin knockout allele (Fxn^tm1Mkn), and may be useful in studying Friedreich's Ataxia. Because FVB;B6.Tg(FXN);Fxn- mice were bred onto the FVB/NJ genetic background for one generation, they may also allow larger litters compared to the C57BL/6-congenic Tg(FXN);Fxn- strain (Stock No. 008586).

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Donating Investigator

IMR Colony, The Jackson Laboratory

Genetic overview

Genetic Background	Generation
001800 FVB/NJ 008586 B6.Cg-Tg(FXN)1Sars Fxn^tm1Mkn*/J

Fxn^tm1Mkn

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

Tg(FXN*)1Sars

Allele Type
Transgenic (Inserted expressed sequence, Humanized sequence)

View Genetics

Research Applications

Neurobiology Research

View All Research Applications

Base Price

Starting at:

$2,854.50 Domestic price Cryo Recovery

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Details

Detailed Description

FVB;B6.Tg(FXN);Fxn- mice (Stock No. 018299) harbor the the FXN*500GAA transgene (Tg(FXN)1Sars) and frataxin knockout allele (Fxn^tm1Mkn), and may be useful in studying Friedreich's Ataxia. Because FVB;B6.Tg(FXN);Fxn- mice were bred onto the FVB/NJ genetic background for one generation, they may also allow larger litters compared to the C57BL/6-congenic Tg(FXN);Fxn- strain (Stock No. 008586). Mice hemizygous for the transgene and homozygous for the knockout allele (FVB;B6.Tg(FXN);Fxn-/-) are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities.

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. It should be noted that the phenotype of FVB;B6.Tg(FXN);Fxn- mice (Stock No. 018299) could vary from that originally described for the C57BL/6-congenic Tg(FXN);Fxn- strain (Stock No. 008586). We will modify the strain description for FVB;B6.Tg(FXN);Fxn- mice if necessary as published results become available.

To date (November 2015) the phenotype of the C57BL/6-congenic Tg(FXN);Fxn- strain (Stock No. 008586) lists that mice hemizygous for the transgene and homozygous for the knockout allele (Tg(FXN);Fxn-/-) are viable, normal in size and do not display any gross physical or behavioral abnormalities. The donating investigator reports that no other behavioral or histological phenotype has been observed. FISH and karyotyping shows one copy of the transgene inserted on chromosome 5.

Development

FVB;B6.Tg(FXN);Fxn- mice (Stock No. 018299) harbor the the FXN*500GAA transgene (Tg(FXN)1Sars) and frataxin knockout allele (Fxn^tm1Mkn) from Stock No. 008586.

The FVB;B6.Tg(FXN);Fxn- strain (Stock No. 018299) was created by first breeding FVB/NJ females (Stock No. 001800) with C57BL/6J-congenic males hemizygous for the FXN*500GAA transgene and homozygous for the frataxin knockout allele (Tg(FXN);Fxn-/- ; from Stock No. 008586). The resulting F1 offspring ([FVBxB6]F1.HEMI;HET and [FVBxB6]F1.NCar;HET) were then bred together to produce F2 animals. The resulting F2 animals were bred together to generate the next intercross generation (F3). The strain was then maintained by continued intercrossing of FVB;B6.HEMI;HET, FVB;B6.NCar;HET, FVB;B6.HEMI;HOM, and/or FVB;B6.NCar;HOM.

The FXN*500GAA transgene, created by Dr. Joseph Sarsero, was designed with a bacterial artificial chromosome (BAC) containing the entire human frataxin (FXN) gene with an interrupted 500 GAA trinucleotide repeat inserted into intron 1. FISH and karyotyping of mice from founder line 1 shows one copy of the transgene inserted on chromosome 5.

The frataxin knockout allele (Frda^- or Frda^del4) was designed by Drs. Michel Koenig and Helene Puccio by replacing exon 4 of the frataxin gene on chromosome 19 with a loxP-flanked PGK-neomycin resistance cassette.

Expression Data

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

Control Suggestions

Noncarrier (Noncarrier from the colony)

Approximate Controls

An approximate genetic background control is (C57BL/6J x FVB/NJ)F1 hybrid mice (Stock No. 019019). This F1 is created by breeding C57BL/6J females (Stock No. 000664) with FVB/NJ males (Stock No. 001800).

Additional Information

Considerations for Choosing Controls

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)
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*)1Sars

Allele Symbol: Tg(FXN*)1Sars

Allele Name	transgene insertion 1, Joseph Sarsero
Allele Type	Transgenic (Inserted expressed sequence, Humanized sequence)
Allele Synonym(s)
Gene Symbol and Name	Tg(FXN*)1Sars, transgene insertion 1, Joseph Sarsero
Gene Synonym(s)
Promoter	FXN, frataxin, human
Expressed Gene	FXN, frataxin, human
Strain of Origin	C57BL/6J
Chromosome	5
Molecular Note	The transgene contained a bacterial artificial chromosome (BAC) with the entire human frataxin (FXN) gene with a 500 GAA trinucleotide repeat inserted into intron 1. Line 1 contained a single copy of the transgene inserted into chromosome 5.

Disease/Phenotype

Disease Terms

Research Areas By Phenotype

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

Neurobiology Research
- Friedreich's Ataxia
- Ataxia (Movement) Defects
- Neuromuscular defects

Mammalian Phenotype Terms by Genotype

References

Additional - Fxn^tm1Mkn related

Additional - Tg(FXN*)1Sars related

Technical Support

CONTACT TECHNICAL SUPPORT

Genotyping Protocols
- Standard PCR:Laragen
- Standard PCR:Generic Human FXN
- Standard PCR:Fxnalternate3
- Genotyping resources and troubleshooting
Breeding Considerations

The FVB;B6.Tg(FXN);Fxn- strain (Stock No. 018299) was created by first breeding FVB/NJ females (Stock No. 001800) with C57BL/6J-congenic males hemizygous for the FXN*500GAA transgene and homozygous for the frataxin knockout allele (Tg(FXN);Fxn-/- ; from Stock No. 008586). The resulting F1 offspring ([FVBxB6]F1.HEMI;HET and [FVBxB6]F1.NCar;HET) were then bred together to produce F2 animals. The resulting F2 animals were bred together to generate the next intercross generation (F3). The strain was then maintained by continued intercrossing of mice hemizygous for the transgene and heterozygous for the knockout allele (Tg(FXN);Fxn+/-) with mice wildtype (noncarrier) for the transgene and heterozygous for knockout allele (Fxn+/-).
- Additional Breeding and Husbandry Support
Mating System
- See "Breeding Considerations"
- Hemizygous for Tg(FXN)1Sars, Homozygous for Fxn x Noncarrier, Heterozygous for Fxn (or reciprocal
Citation
When using the FVB;B6.Tg(FXN);Fxn- mouse strain in a publication, please cite the originating article(s) and include JAX stock #018299 in your Materials and Methods section.

Animal Health Reports

Facility Barrier Level Descriptions

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

G3A (Intermediate)

Pricing & Availability

Cryo Recovery

Domestic
International

Live Mouse

Age

Genotype

Price

weeks

Cryorecovery - Pricing

Service/Product	Description	Price
	Hemizygous or non carrier for Tg(FXN*)1Sars and heterozygous for Fxn<tm1Mkn>

Related Products and Services

Frozen Mouse Embryo	STOCK Tg(FXN*)1Sars Fxn<tm1Mkn>/J	$2595.00
Frozen Mouse Embryo	STOCK Tg(FXN*)1Sars Fxn<tm1Mkn>/J	$2595.00
Frozen Mouse Embryo	STOCK Tg(FXN*)1Sars Fxn<tm1Mkn>/J	$3373.50
Frozen Mouse Embryo	STOCK Tg(FXN*)1Sars Fxn<tm1Mkn>/J	$3373.50

Payment Terms and Conditions

Terms are granted by individual review and stated on the customer invoice(s) and account statement. These transactions are payable in U.S. currency within the granted terms. Payment for services, products, shipping containers, and shipping costs that are rendered are expected within the payment terms indicated on the invoice or stated by contract. Invoices and account balances in arrears of stated terms may result in The Jackson Laboratory pursuing collection activities including but not limited to outside agencies and court filings.

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

Licensing Information

Phone: 207-288-6470

Email: TechTran@jax.org

Also Known As:FVB;B6.Tg(FXN);Fxn-

Donating Investigator

Genetic overview

Research Applications

Base Price

Detailed Description

Development

Expression Data

Control Suggestions

Approximate Controls

Additional Information

Fxntm1Mkn

Allele Symbol: Fxntm1Mkn

Tg(FXN*)1Sars

Allele Symbol: Tg(FXN*)1Sars

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 - Fxntm1Mkn related

Additional - Tg(FXN*)1Sars related

Genotyping Protocols

Breeding Considerations

Mating System

Citation

Animal Health Reports

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

Live Mouse

Cryorecovery - Pricing

Related Products and Services

Payment Terms and Conditions

The Jackson Laboratory's Genotype Promise

Terms Of Use

Licensing Information

Fxn^tm1Mkn

Allele Symbol: Fxn^tm1Mkn

Additional - Fxn^tm1Mkn related