Strain Name:
129S1.Cg-Fbn1tm1.1Itl/RmzMmnc
Stock Number:
069518-UNC
Citation ID:
RRID:MMRRC_069518-UNC
Other Names:
Fbn1lox

Strain Information

Fbn1tm1.1Itl
Name: fibrillin 1; targeted mutation 1.1, inGenious Targeting Laboratory
Synonyms: Fbn1Lox
Type: Allele
Species: Mus musculus (mouse)
Chromosome: 6
Alteration at locus: Conditional
Fbn1
Name: fibrillin 1
Synonyms: Fib-1
Type: Gene
Species: Mus musculus (mouse)
Chromosome: 2
Alteration at locus: Conditional
NCBI: 14118
HGNC: HGNC:3603
Homologene: 30958
Genetic Alterations
Conditional-ready allele where a loxP site was inserted upstream of exon 1 of the Fbn1 gene. An FRT-flanked neo cassette with a 3' loxP site was inserted downstream of exon 1. Flp-mediated recombination removed the neo cassette and left exon 1 floxed.
Genotype Determination
  • Genotyping Protocol(s)
  • Center protocol and contact for technical support will be shipped with mice.
  • ES Cell Line
    R1 derived from (129X1/SvJ x 129S1/Sv)F1-Kitl+
    Phenotype
    No observable differences between wild-type and mutant mice. However, when crossed with CMV-Cre mice present similarly to Fbn1-null mice, the animals die before reaching one month of age due to ruptured aortic aneurysm, impaired pulmonary function, or diaphragmatic collapse. Post mortem examinations show aortic aneurysms with hemothorax or hemopericardium, severely affected lungs with abnormal enlargement of the alveolar space, and abdominal organs within the chest cavity. Mice also display grossly malformed and elongated ribs, seemingly thinner intercostal muscles, and unusually fragile skin and internal organs upon manipulation of the carcasses.
    MeSH Terms
    • Animals
    • Bone Density/genetics
    • Cell Differentiation
    • Fibrillin-1
    • Fibrillins
    • Gene Knockdown Techniques
    • Gene Order
    • Gene Targeting/methods
    • Genotype
    • Mice
    • Mice, Knockout
    • Microfibrils/genetics
    • Microfibrils/metabolism
    • Microfilament Proteins/genetics
    • Mutation
    • Osteoblasts/cytology
    • Osteoblasts/metabolism
    • Osteogenesis/genetics
    • Phenotype
    Strain Development
    R1 embryonic stem cells derived from (129X1/SvJ x 129S1/Sv)F1-Kitl+ were selected for G418 resistance and subjected to Southern blotting to validate proper integration of the targeting vector into the Fbn1 locus. Following microinjection into C57BL/6J blastocysts, chimeric mice were bred to monitor germline transmission. Fbn1fneo mice were crossed with FLPeR mice (129S4/SvJaeSor-Gt(ROSA)26Sortm1(FLP1)Dym/J - RRID:IMSR_JAX:003946) to excise the neo cassette and create Fbn1lox mice. The strain was backcrossed to 129S1/SvImJ (RRID:IMSR_JAX:002448) to the 10th generation.
    Suggested Control Mice
    129S1/SvImJ
    MMRRC Genetic QC Summary
    The MMRRC Centers have developed a genetic QC pipeline using MiniMUGA array genotyping to provide additional information on strain backgrounds for MMRRC congenic and inbred strains. For more information on when data may be available, or to request genotyping for a strain of interest, please contact mmrrc@med.unc.edu. Older strains may not have this information.
    • Cardiovascular
    • Dermatology
    • Models for Human Disease
    Donor
    Francesco Ramirez, Ph.D., Icahn School of Medicine at Mount Sinai.
    Primary Reference

    Cook JR, Smaldone S, Cozzolino C, del Solar M, Lee-Arteaga S, Nistala H, Ramirez F. Generation of Fbn1 conditional null mice implicates the extracellular microfibrils in osteoprogenitor recruitment. Genesis. 2012 Aug;50(8):635-41. doi: 10.1002/dvg.22022. Epub 2012 Mar 31. (Medline PMID: 22374917)

    Colony and Husbandry Information

    Colony Surveillance Program and Current Health Reports

    Mice recovered from a cryo-archive will have health surveillance performed on recipient females. Health reports will be provided prior to shipment. If you require additional health status information, please email mmrrc_health@med.unc.edu .
    Coat Color
    Agouti
    Eye
    Black
    MMRRC Breeding System
    Backcross
    Generation
    N10 (129S1/SvImJ), F?
    Overall Breeding Performance
    Excellent
    Viability and Fertility: Female Male Comments
    Homozygotes are viable: Yes Yes
    Homozygotes are fertile: Yes Yes
    Heterozygotes are fertile: Yes Yes
    Age Reproductive Decline: 10 to 12 months Greater than 12 months
    Average litter size
    7 to 9
    Recommended wean age
    3 Weeks
    Average Pups Weaned
    7 to 9

    Order Request Information

    Limited quantities of breeder mice (recovered litter) are available from a cryoarchive; recovered litter usually available to ship in 3 to 4 months.

    Cryopreserved material may be available upon request, please inquire to mmrrc@med.unc.edu for more information.

    Distribution of this strain requires submission of the MMRRC Conditions of Use (COU). A link to the COU web form will be provided via email after an order has been placed; the form should be completed then or the email forwarded to your institutional official for completion.

    The donor or their institution limits the distribution to non-profit institutions only.

    Additional charges may apply for any special requests. Shipping costs are in addition to the basic distribution/resuscitation fees. Information on shipping costs and any additional charges will be provided by the supplying MMRRC facility.

    Click button to Request this one strain. (Use the MMRRC Catalog Search to request more than one strain.)
    MMRRC Item # Description Distribution Fee / Unit (US $)
    *Shipping & Handling not included*
    Units Notes
    069518-UNC-RESUS Litter recovered from cryo-archive $2,914.00 / Non-Profit Litter Recovered litter4; additional fees for any special requests.
    Cryopreserved material may be available upon request, please inquire to mmrrc@med.unc.edu for more information.

    1 The distribution fee covers the expense of rederiving mice from a live mouse; you will receive the resulting litter. The litter will contain at minimum one mutant carrier; the actual number of animals and the gender and genotype ratios will vary. (Typically, multiple breeder pairs can be established from the recovered litter.) Prior to shipment, the MMRRC will provide information about the animals recovered. If you anticipate or find that you need to request specific genotypes, genders or quantities of mice in excess of what is likely from a resuscitated litter, you may discuss available options and pricing with the supplying MMRRC facility.

    2 An aliquot contains a sufficient number of embryos (in one or more vials or straws and based on the transfer success rate of the MMRRC facility) to transfer into one to three recipients. The MMRRC makes no guarantee concerning embryo transfer success experienced in the recipient investigator's laboratory. Neither gender nor genotype ratios are guaranteed.

    3 An aliquot is one straw or vial with sufficient sperm to recover at least one litter of mice, as per provided protocols, when performed at the MMRRC facility. The MMRRC makes no guarantee concerning the success of these procedures when performed outside the MMRRC facilities.

    4 The distribution fee covers the expense of resuscitating mice from the cryo-archive; you will receive the resulting litter. The litter will contain at minimum one mutant carrier; the actual number of animals and the gender and genotype ratios will vary. (Typically, multiple breeder pairs can be established from the recovered litter.) Prior to shipment, the MMRRC will provide information about the animals recovered. If you anticipate or find that you need to request specific genotypes, genders or quantities of mice in excess of what is likely from a resuscitated litter, you may discuss available options and pricing with the supplying MMRRC facility.

    To request material from the MMRRC: Please fill out our on-line request form (accessible from the catalog search results page, or click the Request this Strain button in the fees section). If you have questions or need assistance completing this form, you may call Customer Service at (800) 910-2291 (in USA or Canada) or (530) 757-5710 (international calls). Before you call, please have with you: the MMRRC item number, quantity needed, Bill-to and Ship-to contact information.