Strain Name:

B6.D2-Fscn2ahl8/4Kjn

Stock Number:

009629

Order this mouse

Availability:

Cryopreserved - Ready for recovery

This congenic is valuable for studying hearing loss related to FSCN2 function.

Description

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Strain Information

Type Congenic; Mutant Strain;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Mating SystemHomozygote x Homozygote         (Female x Male)   21-FEB-13
Specieslaboratory mouse
GenerationN6F4pN1
Generation Definitions

Description
This congneic strain has the Fscn2ahl8 allele backcrossed from DAB/2J onto C57BL/6J.

Development
This C57BL/6J subcongenic line with the DBA/2J-derived Fscn2ahl8 mutation was developed by first crossing mice of the B6.D2-Chr11D/LusJ congenic strain (Davis et al., 2005) with C57BL/6J mice. The hybrid progeny from this cross were then mated with C57BL/6J mice, and the resulting backcross progeny were genotyped for five DNA microsatellite markers spanning the introgressed region of the B6.D2-Chr11D/LusJ congenic strain. Backcross mice that were homozygous for C57BL/6J alleles at markers D11Mit90, D11Mit360, D11Mit338, and D11Mit203, and heterozygous for C57BL/6J and DBA/2J alleles at D11Mit104 were selected and interbred. The progeny from these matings were genotyped and those that were confirmed to be homozygous for C57BL/6J alleles at D11Mit90 (70.5 Mb position of Chr 11, GRCm38), D11Mit360 (103.4 Mb), D11Mit338 (115.5 Mb), and D11Mit203 (116.4 Mb), and homozygous for DBA/2J alleles at D11Mit104 (119.3) and Fscn2 (120.4 Mb) were interbred to produce this homozygous subcongenic line. This strain is maintained by sibling inbreeding homozygotes.

Control Information

  Control
   000664 C57BL/6J
 
  Considerations for Choosing Controls

Related Strains

Strains carrying   Fscn2ahl8 allele
000671   DBA/2J
View Strains carrying   Fscn2ahl8     (1 strain)

Strains carrying other alleles of Fscn2
012438   D2.B6-Fscn2+/Kjn
012440   DBA/2J-Tg(RP24-180N9)2Kjn/Kjn
View Strains carrying other alleles of Fscn2     (2 strains)

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Retinitis Pigmentosa 30; RP30   (FSCN2)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

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

Fscn2ahl8/Fscn2ahl8

        DBA/2J
  • hearing/vestibular/ear phenotype
  • abnormal auditory brainstem response
    • although DBA/2J is homozygous for both Cdh23 and ahl8, through linkage analysis with CAST/Ei, which is wild-type at Cdh23, ahl8 is shown to contribute up to 37% of the 16 kHz ABR threshold variation in 13 week old homozygotes   (MGI Ref ID J:139223)
    • increased or absent threshold for auditory brainstem response
      • increased ABR threshold   (MGI Ref ID J:162868)
  • decreased inner hair cell stereocilia number
    • hair bundles appear normal at 2 weeks of age but are progressively lost between 1 and 6 months of age   (MGI Ref ID J:162868)
  • decreased outer hair cell stereocilia number
    • degeneration occurs earliest here   (MGI Ref ID J:162868)
  • increased susceptibility to age-related hearing loss
    • hearing loss is progressive and shows a much earlier onset than in C57BL/6J, with average 16 kHz ABR thresholds approximately 50 dB higher at 10 weeks of age than in 30 week old C57BL/6J   (MGI Ref ID J:139223)
    • hearing appears normal at 2 weeks of age but is progressively lost, based upon measurement of auditory brainstem response between 1 and 6 months of age   (MGI Ref ID J:162868)
  • nervous system phenotype
  • decreased inner hair cell stereocilia number
    • hair bundles appear normal at 2 weeks of age but are progressively lost between 1 and 6 months of age   (MGI Ref ID J:162868)
  • decreased outer hair cell stereocilia number
    • degeneration occurs earliest here   (MGI Ref ID J:162868)
View Research Applications

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

Fscn2ahl8 related

Sensorineural Research
Hearing Defects
      Age related hearing loss

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Fscn2ahl8
Allele Name age related hearing loss 18
Allele Type Spontaneous
Common Name(s) Fscn2R109H;
Strain of OriginDBA/2J
Gene Symbol and Name Fscn2, fascin homolog 2, actin-bundling protein, retinal (Strongylocentrotus purpuratus)
Chromosome 11
Gene Common Name(s) C630046B20Rik; RFSN; RIKEN cDNA C630046B20 gene; RP30; age related hearing loss 8; ahl8;
General Note ahl8 interacts with Cdh23 on chromosome 10.
Molecular Note G to A transition located in exon 1 and is of predicted to cause a nonsynonymous amino acid change of arginine to histidine at position 109 (R109H). The known genealogy of the DBA-related strains and genotyping analysis of archived DBA/2J DNA samples indicate that this allele occurred in the DBA/2J lineage between 1951 (when it was separated from the DBA/2N lineage) and 1975. [MGI Ref ID J:139223] [MGI Ref ID J:162868]

Genotyping

Genotyping Information

Genotyping Protocols

Fscn2+, Pyrosequencing


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Additional References

Fscn2ahl8 related

Johnson KR; Longo-Guess C; Gagnon LH; Yu H; Zheng QY. 2008. A locus on distal chromosome 11 (ahl8) and its interaction with Cdh23 ahl underlie the early onset, age-related hearing loss of DBA/2J mice. Genomics 92(4):219-25. [PubMed: 18662770]  [MGI Ref ID J:139223]

Perrin BJ; Strandjord DM; Narayanan P; Henderson DM; Johnson KR; Ervasti JM. 2013. beta-Actin and Fascin-2 Cooperate to Maintain Stereocilia Length. J Neurosci 33(19):8114-21. [PubMed: 23658152]  [MGI Ref ID J:197137]

Shin JB; Longo-Guess CM; Gagnon LH; Saylor KW; Dumont RA; Spinelli KJ; Pagana JM; Wilmarth PA; David LL; Gillespie PG; Johnson KR. 2010. The R109H variant of fascin-2, a developmentally regulated actin crosslinker in hair-cell stereocilia, underlies early-onset hearing loss of DBA/2J mice. J Neurosci 30(29):9683-94. [PubMed: 20660251]  [MGI Ref ID J:162868]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

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

Colony Maintenance

Mating SystemHomozygote x Homozygote         (Female x Male)   21-FEB-13

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


Pricing for USA, Canada and Mexico shipping destinations View International Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $2525.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $3283.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Control Information

  Control
   000664 C57BL/6J
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

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.


See Terms of Use tab for General Terms and Conditions


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.
Ordering Information
JAX® Mice
Surgical and Preconditioning Services
JAX® Services
Customer Services and Support
Tel: 1-800-422-6423 or 1-207-288-5845
Fax: 1-207-288-6150
Technical Support Email Form

Terms of Use

Terms of Use


General Terms and Conditions


Contact information

General inquiries regarding Terms of Use

Contracts Administration

phone:207-288-6470

JAX® Mice, Products & Services Conditions of Use

"MICE" means mouse strains, their progeny derived by inbreeding or crossbreeding, unmodified derivatives from mouse strains or their progeny supplied by The Jackson Laboratory ("JACKSON"). "PRODUCTS" means biological materials supplied by JACKSON, and their derivatives. "RECIPIENT" means each recipient of MICE, PRODUCTS, or services provided by JACKSON including each institution, its employees and other researchers under its control. MICE or PRODUCTS shall not be: (i) used for any purpose other than the internal research, (ii) sold or otherwise provided to any third party for any use, or (iii) provided to any agent or other third party to provide breeding or other services. Acceptance of MICE or PRODUCTS from JACKSON shall be deemed as agreement by RECIPIENT to these conditions, and departure from these conditions requires JACKSON's prior written authorization.

No Warranty

MICE, PRODUCTS AND SERVICES ARE PROVIDED “AS IS”. JACKSON EXTENDS NO WARRANTIES OF ANY KIND, EITHER EXPRESS, IMPLIED, OR STATUTORY, WITH RESPECT TO MICE, PRODUCTS OR SERVICES, INCLUDING ANY IMPLIED WARRANTY OF MERCHANTABILITY OR FITNESS FOR A PARTICULAR PURPOSE, OR ANY WARRANTY OF NON-INFRINGEMENT OF ANY PATENT, TRADEMARK, OR OTHER INTELLECTUAL PROPERTY RIGHTS.

In case of dissatisfaction for a valid reason and claimed in writing by a purchaser within ninety (90) days of receipt of mice, products or services, JACKSON will, at its option, provide credit or replacement for the mice or product received or the services provided.

No Liability

In no event shall JACKSON, its trustees, directors, officers, employees, and affiliates be liable for any causes of action or damages, including any direct, indirect, special, or consequential damages, arising out of the provision of MICE, PRODUCTS or services, including economic damage or injury to property and lost profits, and including any damage arising from acts or negligence on the part of JACKSON, its agents or employees. Unless prohibited by law, in purchasing or receiving MICE, PRODUCTS or services from JACKSON, purchaser or recipient, or any party claiming by or through them, expressly releases and discharges JACKSON from all such causes of action or damages, and further agrees to defend and indemnify JACKSON from any costs or damages arising out of any third party claims.

MICE and PRODUCTS are to be used in a safe manner and in accordance with all applicable governmental rules and regulations.

The foregoing represents the General Terms and Conditions applicable to JACKSON’s MICE, PRODUCTS or services. In addition, special terms and conditions of sale of certain MICE, PRODUCTS or services may be set forth separately in JACKSON web pages, catalogs, price lists, contracts, and/or other documents, and these special terms and conditions shall also govern the sale of these MICE, PRODUCTS and services by JACKSON, and by its licensees and distributors.

Acceptance of delivery of MICE, PRODUCTS or services shall be deemed agreement to these terms and conditions. No purchase order or other document transmitted by purchaser or recipient that may modify the terms and conditions hereof, shall be in any way binding on JACKSON, and instead the terms and conditions set forth herein, including any special terms and conditions set forth separately, shall govern the sale of MICE, PRODUCTS or services by JACKSON.


(6.8)