Strain Name:

C57BL/6J-Lgi4clp/J

Stock Number:

000974

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Availability:

Cryopreserved - Ready for recovery

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

Former Names C57BL/6J-clp/J    (Changed: 14-MAR-06 )
C57BL/6J-clp/clp    (Changed: 15-DEC-04 )
Type Coisogenic; Mutant Strain; Spontaneous Mutation;
Additional information on Genetically Engineered and Mutant Mice.
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Specieslaboratory mouse

Control Information

  Control
   Untyped from the colony
   000664 C57BL/6J
 
  Considerations for Choosing Controls

Phenotype

Phenotype Information

View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Lgi4clp/Lgi4clp

        C57BL/6J
  • mortality/aging
  • partial postnatal lethality
    • homozygotes with severe forelimb abnormalities often die within 2 to 3 days after birth due to difficulties in positioning themselves for suckling and thus do not get enough nourishment, however if they do survive the first few weeks, their condition usually begins to improve and some have a relatively normal lifespan   (MGI Ref ID J:11392)
  • growth/size/body phenotype
  • decreased body size
    • adults with forelimb abnormalities are almost always smaller than controls   (MGI Ref ID J:11392)
  • nervous system phenotype
  • abnormal myelination
    • all homozygotes have delayed and abnormal myelination of the peripheral nervous system but not the central nervous system   (MGI Ref ID J:11392)
    • failure of myelination in some fibers leads to persistent promyelin fibers with small axons, especially in muscles that normally have small axons   (MGI Ref ID J:11392)
    • neuron-Schwann cell cocultures from E13 dissociated dorsal root ganglia do not myelinate   (MGI Ref ID J:105260)
    • delay in myelination and a persistence of some axons and Schwann cells in the promyelin configuration throughout life   (MGI Ref ID J:24743)
  • abnormal nervous system morphology
    • exhibit a variable increase in peripheral nerve fasciculation and an occasional mild increase in endoneural connective tissue, however no differences in the number of Schwann cells or any evidence of denervation atrophy   (MGI Ref ID J:11392)
    • all peripheral nerves are hypotrophic   (MGI Ref ID J:105260)
    • abnormal axon morphology
      • the axons that are enclosed by promyelin fibers have smaller axon calibers than those of myelinated fibers   (MGI Ref ID J:11392)
      • abnormal myelin sheath morphology
        • variable degree of generalized hypomyelination, with peripheral myelin sheaths thinner relative to axonal size than in controls   (MGI Ref ID J:11392)
        • in some mutants, the ratio of myelin sheath thickness to axon caliber is reduced   (MGI Ref ID J:24743)
    • abnormal sciatic nerve morphology
      • hypertrophy of the sciatic nerve   (MGI Ref ID J:105260)
  • limbs/digits/tail phenotype
  • abnormal limb morphology
    • exhibit an arthrogryposis-like phenotype affecting the forelimbs, and in severe cases, also the hindlimbs   (MGI Ref ID J:105260)
    • abnormally flexed joints can be extended early in life but fixed contractures often develop with age   (MGI Ref ID J:11392)
    • abnormal digit morphology
      • fingers are flexed   (MGI Ref ID J:24743)
    • abnormal forelimb morphology
      • flexed forelimbs (fingers, wrists, shoulders), elbows rotated inwards   (MGI Ref ID J:24743)
      • forelimbs with abnormal postures are held flexed at one or more of the shoulder, wrist or digital joints but extended at the elbow, resulting in the forelimbs being pointed toward the hindlimbs   (MGI Ref ID J:11392)
      • decreased diameter of radius
        • radii are thinner and somewhat straighter than in controls, however gross bony landmarks and sections through bones and joints appear normal   (MGI Ref ID J:11392)
      • decreased diameter of ulna
        • ulnae are thinner and somewhat straighter than in controls   (MGI Ref ID J:11392)
    • abnormal hindlimb morphology
      • hindlimbs are more subtly affected than forelimbs, with the most common abnormality a mild, usually symmetrical, weakness of hindpaw dorsiflexion   (MGI Ref ID J:11392)
  • behavior/neurological phenotype
  • abnormal locomotor behavior
    • homozygotes with bilateral forelimb abnormalities have a difficult time from a very young age in moving around the cage and slither around the cage on the undersurface of their muzzles   (MGI Ref ID J:11392)
    • develop a more pronounced waddle as grow older   (MGI Ref ID J:11392)
  • abnormal maternal nurturing
    • maternal abilities are rarely good in females with severe forelimb abnormalities   (MGI Ref ID J:11392)
  • abnormal posture   (MGI Ref ID J:105260)
    • abnormal forelimb joint postures seen within 1-2 days after birth   (MGI Ref ID J:11392)
    • postural deformity (waiter's tip posture) of one or both forelimbs in which the fingers are flexed and the wrist and shoulder may also be flexed and the elbow rotated inward   (MGI Ref ID J:24743)
  • abnormal sexual interaction
    • males breed poorly, however females are able to breed successfully   (MGI Ref ID J:11392)
  • impaired righting response
    • unable to extend an affected forelimb above the head as part of a righting response when turned from its back onto one side   (MGI Ref ID J:11392)
  • skeleton phenotype
  • abnormal long bone morphology   (MGI Ref ID J:11392)
    • decreased diameter of radius
      • radii are thinner and somewhat straighter than in controls, however gross bony landmarks and sections through bones and joints appear normal   (MGI Ref ID J:11392)
    • decreased diameter of ulna
      • ulnae are thinner and somewhat straighter than in controls   (MGI Ref ID J:11392)
  • muscle phenotype
  • *normal* muscle phenotype
    • do not exhibit any histological abnormalities in skeletal muscle or any reductions in large limb muscle mass   (MGI Ref ID J:11392)

Lgi4clp/Lgi4clp

        involves: C57BL/6J
  • nervous system phenotype
  • demyelination
    • in the sciatic nerve at P12   (MGI Ref ID J:158973)
View Research Applications

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

Lgi4clp related

Neurobiology Research
Myelination Defects

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Lgi4clp
Allele Name claw paw
Allele Type Spontaneous
Strain of OriginC57BL/6J-Lep/+
Gene Symbol and Name Lgi4, leucine-rich repeat LGI family, member 4
Chromosome 7
Gene Common Name(s) LGIL3; claw paw; clp;
General Note This mutation arose at The Jackson Laboratory in 1977. The pathology of homozygous mutant mice differs from other myelin deficient mutants in that there is no obvious increase in Schwann cells and that the axons that persist in promyelin fibers are smaller (J:11392).
Molecular Note A CTCT repeat located six base pairs 5' to exon 4 is replaced with a 225-bp repetitive sequence which is 100% homologous to a repeat element located 797 bp upstream. This interferes with the third intron 3' splice site resulting in exon3 spliced to exon5. The resulting protein, lacking exon 4, is not secreted as is the wild-type allele. [MGI Ref ID J:105260]

Genotyping

Genotyping Information


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Additional References

Henry EW; Eicher EM; Sidman RL. 1991. The mouse mutation claw paw: forelimb deformity and delayed myelination throughout the peripheral nervous system. J Hered 82(4):287-94. [PubMed: 1652607]  [MGI Ref ID J:11392]

Lgi4clp related

Bermingham JR Jr; Shearin H; Pennington J; O'Moore J; Jaegle M; Driegen S; van Zon A; Darbas A; Ozkaynak E; Ryu EJ; Milbrandt J; Meijer D. 2006. The claw paw mutation reveals a role for Lgi4 in peripheral nerve development. Nat Neurosci 9(1):76-84. [PubMed: 16341215]  [MGI Ref ID J:105260]

Bermingham JR Jr; Shumas S; Whisenhunt T; Sirkowski EE; O'Connell S; Scherer SS; Rosenfeld MG. 2002. Identification of genes that are downregulated in the absence of the POU domain transcription factor pou3f1 (Oct-6, Tst-1, SCIP) in sciatic nerve. J Neurosci 22(23):10217-31. [PubMed: 12451123]  [MGI Ref ID J:80437]

Darbas A; Jaegle M; Walbeehm E; van den Burg H; Driegen S; Broos L; Uyl M; Visser P; Grosveld F; Meijer D. 2004. Cell autonomy of the mouse claw paw mutation. Dev Biol 272(2):470-82. [PubMed: 15282162]  [MGI Ref ID J:92324]

Henry E; Sidman R. 1987. Claw paw, a new mutation affecting peripheral nerves Mouse News Lett 78:46.  [MGI Ref ID J:24743]

Henry EW; Eicher EM; Sidman RL. 1991. The mouse mutation claw paw: forelimb deformity and delayed myelination throughout the peripheral nervous system. J Hered 82(4):287-94. [PubMed: 1652607]  [MGI Ref ID J:11392]

Koszowski AG; Owens GC; Levinson SR. 1998. The effect of the mouse mutation claw paw on myelination and nodal frequency in sciatic nerves. J Neurosci 18(15):5859-68. [PubMed: 9671673]  [MGI Ref ID J:49329]

Niemann S; Sidman RL; Nave KA. 1998. Evidence against altered forms of MAG in the dysmyelinated mouse mutant claw paw. Mamm Genome 9(11):903-4. [PubMed: 9799842]  [MGI Ref ID J:50579]

Ozkaynak E; Abello G; Jaegle M; van Berge L; Hamer D; Kegel L; Driegen S; Sagane K; Bermingham JR Jr; Meijer D. 2010. Adam22 is a major neuronal receptor for Lgi4-mediated Schwann cell signaling. J Neurosci 30(10):3857-64. [PubMed: 20220021]  [MGI Ref ID J:158973]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

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

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* $3300.00
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 of Strains Needing Progeny Testing
    At least two untested males and two untested females (two pairs) will be recovered (eight or more mice is typical). The total number of animals provided, their gender and genotype will vary. Untested animals typically are available to ship between 10 and 14 weeks from the date of your order. If the first recovery attempt is unsuccessful, a second recovery will be done, extending the overall recovery time to approximately 25 weeks. Progeny testing is required to identify the genotype of mice of this strain, as a genotyping assay is not available. This type of testing involves breeding the recovered animals and assessing the phenotype of the offspring in order to identify animals carrying the mutation of interest. We can perform the progeny testing for you as a service or we can ship all recovered animals to you for progeny testing at your facility. If you perform the progeny testing, there is no guarantee that a carrier will be identified. If we perform progeny testing as a service, additional breeding time will be required. In this case, when a male and female (one pair) are identified that carry the mutation, they and their offspring will be shipped. Delivery time for strains requiring progeny testing often exceeds 25 weeks and may take 12 months or more due to the difficulties in breeding some strains. The progeny testing cost is in addition to the recovery cost and is based on the number of boxes used and the time taken to produce the mice identified as carrying the mutation.
    Please note that identified pairs may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation of the strain. Mating schemes are sometimes modified for successful cryopreservation.

    Please contact Customer Service for more information on the cost of progeny testing for a strain, tel: 1-800-422-6423 or 1-207-288-5845 (from any location). 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* $4290.00
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 of Strains Needing Progeny Testing
    At least two untested males and two untested females (two pairs) will be recovered (eight or more mice is typical). The total number of animals provided, their gender and genotype will vary. Untested animals typically are available to ship between 10 and 14 weeks from the date of your order. If the first recovery attempt is unsuccessful, a second recovery will be done, extending the overall recovery time to approximately 25 weeks. Progeny testing is required to identify the genotype of mice of this strain, as a genotyping assay is not available. This type of testing involves breeding the recovered animals and assessing the phenotype of the offspring in order to identify animals carrying the mutation of interest. We can perform the progeny testing for you as a service or we can ship all recovered animals to you for progeny testing at your facility. If you perform the progeny testing, there is no guarantee that a carrier will be identified. If we perform progeny testing as a service, additional breeding time will be required. In this case, when a male and female (one pair) are identified that carry the mutation, they and their offspring will be shipped. Delivery time for strains requiring progeny testing often exceeds 25 weeks and may take 12 months or more due to the difficulties in breeding some strains. The progeny testing cost is in addition to the recovery cost and is based on the number of boxes used and the time taken to produce the mice identified as carrying the mutation.
    Please note that identified pairs may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation of the strain. Mating schemes are sometimes modified for successful cryopreservation.

    Please contact Customer Service for more information on the cost of progeny testing for a strain, tel: 1-800-422-6423 or 1-207-288-5845 (from any location). 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
   Untyped from the colony
   000664 C57BL/6J
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

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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.
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