Strain Detail Sheet

Strain Name    :

B6.129S4(C3)-Bgntm1Mfy/Mmmh

Stock Number :

030089-MU

Gene Information

Gene Details [Including genotyping protocols]

(provided by MGI)
Allele Symbol: Bgntm1Mfy
Name: targeted mutation 1, Marian F Young
Chromosome: X
Alteration at locus: Knockout
Gene Symbol: Bgn
Name: biglycan
Chromosome: X
Alteration at locus: Knockout

Genetic Alterations:
neo casette insertion into exon 2 just 5' to the start of translation

Genotype Determination:

ES Cell Line: J1

Strain Description [Including phenotype, strain background, strain development and suggested control mice]

Phenotype

Homozygous phenotype: Age dependent osteoporosis starting in homozygous females after 3 months of age (judged by X-ray and histomorphometry). Mild muscular dystrophy like sydrome (judged by ultrastructure of tissue) thinned skin in phenotype like mild osteogenesis imperfecta (judged by physical weakness and histology).

Hemizygous phenotype: Age dependent osteoporosis starting in hemizygous males after 3 months of age (judged by X-ray and histomorphometry). Mild muscular dystrophy like sydrome (judged by ultrastructure of tissue) thinned skin in phenotype like mild osteogenesis imperfecta (judged by physical weakness and histology).


Mammalian Phenotype Terms:(provided by MGI)      Extend all MPTs
      assigned by genotype

The following phenotype information may relate to one or more alleles on a genetic background differing from this MMRRC strain.
Bgntm1Mfy/-
        involves: 129S4/SvJae * C57BL/6
  • growth/size phenotype
    • decreased body weight (MGI Ref ID J:49647)
      • mutant males display no patterning defects and grow normally until 3 months of age
      • at 6 months, their body weight is significantly reduced; however, this difference disappears by 9 months of age
  • immune system phenotype
    • osteoarthritis (MGI Ref ID J:76203)
      • by 3 months, mutants develop a statistically significant level of osteoarthritis
  • limbs/digits/tail phenotype
    • abnormal femur morphology (MGI Ref ID J:49647)
      • mutants have a wider angle between the femoral neck and the greater trochanter
      • short femur (MGI Ref ID J:49647)
        • at 6 months, femur length is slightly shorter in mutant males
        • femurs become progressively shorter at 9 months of age
    • abnormal long bone diaphysis morphology (MGI Ref ID J:49647)
      • the cortical thickness of the diaphysis is reduced in long bones
    • abnormal long bone epiphysis morphology (MGI Ref ID J:49647)
      • the epiphyseal trabecular structures of long bones are thinner, fewer and poorly connected to each other
    • abnormal long bone metaphysis morphology (MGI Ref ID J:49647)
      • the metaphyseal trabecular structures of long bones are thinner, fewer and poorly connected to each other
      • similar changes in trabecular structures are detected in the vertebrae
  • muscle phenotype
    • abnormal tendon morphology (MGI Ref ID J:76203)
      • relative to wild-type, mutant mice display absence of large collagen fibrils (>190 nm) and a rather uniform fibril diameter range in the quadriceps tendon
  • skeleton phenotype
    • abnormal bone ossification (MGI Ref ID J:76203)
      • at 3 months, mutants knees show a significant ectopic tendon ossification that does not significantly increase with age
      • in contrast, mutant ankles are not significantly different from wild-type at either 3 or 9 months of age
      • males are affected more than females
    • abnormal femur morphology (MGI Ref ID J:49647)
      • mutants have a wider angle between the femoral neck and the greater trochanter
      • short femur (MGI Ref ID J:49647)
        • at 6 months, femur length is slightly shorter in mutant males
        • femurs become progressively shorter at 9 months of age
    • abnormal long bone diaphysis morphology (MGI Ref ID J:49647)
      • the cortical thickness of the diaphysis is reduced in long bones
    • abnormal long bone epiphysis morphology (MGI Ref ID J:49647)
      • the epiphyseal trabecular structures of long bones are thinner, fewer and poorly connected to each other
    • abnormal long bone metaphysis morphology (MGI Ref ID J:49647)
      • the metaphyseal trabecular structures of long bones are thinner, fewer and poorly connected to each other
      • similar changes in trabecular structures are detected in the vertebrae
    • abnormal tendon morphology (MGI Ref ID J:76203)
      • relative to wild-type, mutant mice display absence of large collagen fibrils (>190 nm) and a rather uniform fibril diameter range in the quadriceps tendon
    • abnormal trabecular bone morphology (MGI Ref ID J:49647)
      • mutants show a reduction in the amount and density of trabecular bone, both in epiphyses and in metaphyses
      • trabeculae are distributed over a shorter distance from the growth plate, and cortical thickness is reduced
    • decreased bone mineral content (MGI Ref ID J:49647)
      • mineralizing surface, mineral apposition rate and bone formation rates are all significantly decreased in mutant mice
      • although total ash weight (an indicator of mineral content) is reduced in mutant long bones, there is no significant reduction in bone ash content
      • the mineral crystal size and shape appears normal relative to wild-type
    • decreased compact bone thickness (MGI Ref ID J:49647)
      • cortical thickness is reduced (20%) compared with the reduction in trabecular bone volume (60-70%)
    • decreased osteoblast cell number (MGI Ref ID J:49647)
      • mutants show a reduction in osteoblast numbers and activity
      • in contrast, osteoclast numbers and activity remain normal
    • osteoarthritis (MGI Ref ID J:76203)
      • by 3 months, mutants develop a statistically significant level of osteoarthritis
    • osteoporosis (MGI Ref ID J:49647)
      • mutants show a reduction in bone mass, which becomes more prominent at 6- and 9-months
      • bone volume/total tissue volume, an index of trabecular bone mass, is reduced to below 50% of wild-type values at 3 and 9 months of age

Strain of Origin: C3H

Strain genetic background: C57BL/6

Strain Development: Donor received C3.129S4(B6)-Bgn/Mmmh mice from the MMRRC (stock number 000040-MU) several years ago and bred them onto the C57BL/6 background.

Suggested Control Mice:

  • Wildtype littermates

Research Applications

  • Cell Biology
  • Developmental Biology
  • Hematology

Strain Origin

Donor: Sean Morrison, Ph.D., University of Michigan

Primary Reference:

Kiel MJ, Radice GL, Morrison SJ. Lack of evidence that hematopoietic stem cells depend on N-cadherin-mediated adhesion to osteoblasts for their maintenance. Cell Stem Cell. 2007 Aug 16;1(2):204-17. (Medline PMID: 18371351)

Colony and Husbandry Information

Special Considerations

None

Health Status Report

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@missouri.edu.

Order Request Information

Availability Level

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

Conditions of Distribution [Including applicable technology transfer agreements]

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.

Fees

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 $)
Units Notes
030089-MU-RESUSLitter recovered from cryo-archive
$2,022.00 / $4,109.00
Non-Profit / For-Profit
Litter Recovered litter1; additional fees for any special requests.
030089-MU-SPERMCryo-preserved spermatozoa
$437.00 / $817.00
Non-Profit / For-Profit
Aliquot Approximate quantity.2

1 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.

2 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.

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

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 above). 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.



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.



The MMRRC is a collaborative effort, funded by grants from DPCPSI of the NIH.

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