Strain Name:

C57BL/6J-Tg(LMNB1)1Yfu/J

Stock Number:

023083

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This transgenic model of adult-onset autosomal-dominant leukodystrophy (ADLD) expresses four times the normal levels of full-length mouse Lmnb1 under the control of the endogenous promoter and regulatory sequences. Mice exhibit cognitive impairment and epilepsy, followed by age-dependent motor deficits.

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 Coisogenic; Mutant Strain; Transgenic;
Additional information on Genetically Engineered and Mutant Mice.
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Specieslaboratory mouse
Generation>10pN1
Generation Definitions
 
Donating Investigator Louis Ptacek,   University of California, San Francisco
Donating Investigator Ying-Hui Fu,   University of California, San Francisco

Description
Adult-onset autosomal-dominant leukodystrophy (ADLD) is a progressive and fatal neurological disorder characterized by early autonomic dysfunction, cognitive impairment, pyramidal tract and cerebellar dysfunction, and white matter loss in the central nervous system. ADLD is caused by duplication of the LMNB1 gene, which results in increased lamin B1 transcripts and protein expression.

This transgenic model of ADLD expresses four times the normal levels of full-length mouse Lmnb1 under the control of the endogenous promoter and regulatory sequences. Expression levels are approximately 4-fold higher compared with wildtype.

Animals are born healthy and are overtly indistinguishable from control littermates, but eventually recapitulate many of the features of ADLD. Mice exhibit cognitive impairment and epilepsy, followed by age-dependent motor deficits.

Substantial spatial memory deficits are observed at 12 months of age in a Morris water maze assay and exhibit impairments in a step-through passive avoidance task, which reflects long-term spatial associative learning and fear memory of an aversive experience. Transgenic animals exhibit progressive motor impairment on 2 different motor tasks, the accelerated rotarod (at 24 months of age) and balance beam (progressing at 12 months).

Frequent behavioral seizures, varying from generalized spasms and tremors to generalized clonic activity with atonia and tail extension are seen. Cortical EEG reveals frequent spontaneous epileptic activity, including epileptiform discharges and seizures. Transgenic mice exhibit an approximately 20-fold increase in the number of interictal spikes. Overexpression results in aberrant myelin formation and axonal degeneration (at 12 months of age), and demyelination (by 24 months).

Development
BAC clone RP23-460J18, carrying the full-length mouse Lmnb1 gene was isolated from a C57BL/6J-background Roswell Park Cancer Institute (RPCI) library. A 177.20 kb fragment encoding the entire locus was introduced to BAC vector pBACe3.6. The vector was subsequently microinjected into C57BL/6J fertilized oocytes. The resultant mice were maintained on a C57BL/6J background by the donating laboratory. This is founder line 1.

Control Information

  Control
   Wild-type from the colony
   000664 C57BL/6J
 
  Considerations for Choosing Controls

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Characteristics of this human disease are associated with transgenes and other mutation types in the mouse.
Leukodystrophy, Demyelinating, Adult-Onset, Autosomal Dominant; ADLD
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Tg(Lmnb1)1Yfu/0

        involves: C57BL/6J
  • behavior/neurological phenotype
  • *normal* behavior/neurological phenotype
    • mice exhibit normal spontaneous avoidance   (MGI Ref ID J:197168)
    • abnormal spatial learning
      • impaired performance in a Morris water maze   (MGI Ref ID J:197168)
      • impaired long-term spatial associated learning and fear memory of an averse experience in a step-through passive avoidance task with no increase in latency to avoid the light and step through the dark chamber where they received an electric shock the day before   (MGI Ref ID J:197168)
    • abnormal spatial reference memory
      • impaired spatial memory retention in a Morris water maze   (MGI Ref ID J:197168)
      • impaired long-term spatial associated learning and fear memory of an averse experience in a step-through passive avoidance task with no increase in latency to avoid the light and step through the dark chamber where they received an electric shock the day before   (MGI Ref ID J:197168)
    • impaired coordination
      • progressive impairment on an accelerating rotarod (at 24, but not 12, months) and balance beam (at 12 months and worse at 24 months)   (MGI Ref ID J:197168)
    • seizures
      • varying from generalized spasms and tremors to generalized clonic activity with atonia and tail extension   (MGI Ref ID J:197168)
      • frequent spontaneous epileptic activity, including epileptiform discharge   (MGI Ref ID J:197168)
      • abnormal spike wave discharge
        • frequent spontaneous epileptic activity, including epileptiform discharge and a 20-fold increase in the number of interictal spikes   (MGI Ref ID J:197168)
      • clonic seizures
        • with atonia and tail extension   (MGI Ref ID J:197168)
      • increased susceptibility to pharmacologically induced seizures
        • when treated with pentylenetetrazol   (MGI Ref ID J:197168)
    • straub tail
      • tail extension   (MGI Ref ID J:197168)
    • tremors   (MGI Ref ID J:197168)
  • nervous system phenotype
  • *normal* nervous system phenotype
    • mice exhibit no obvious inflammation in the brain   (MGI Ref ID J:197168)
    • abnormal myelin sheath morphology
      • outfoldings, extensions and invaginations originating from the myelin sheath   (MGI Ref ID J:197168)
    • abnormal oligodendrocyte physiology
      • frequently dying and degenerating at 24 months   (MGI Ref ID J:197168)
    • axon degeneration
      • disintegration and degradation   (MGI Ref ID J:197168)
    • demyelination
      • in some fibers at 12, but not 24, months   (MGI Ref ID J:197168)
    • hypermyelination
      • in some fibers   (MGI Ref ID J:197168)
    • seizures
      • varying from generalized spasms and tremors to generalized clonic activity with atonia and tail extension   (MGI Ref ID J:197168)
      • frequent spontaneous epileptic activity, including epileptiform discharge   (MGI Ref ID J:197168)
      • abnormal spike wave discharge
        • frequent spontaneous epileptic activity, including epileptiform discharge and a 20-fold increase in the number of interictal spikes   (MGI Ref ID J:197168)
      • clonic seizures
        • with atonia and tail extension   (MGI Ref ID J:197168)
      • increased susceptibility to pharmacologically induced seizures
        • when treated with pentylenetetrazol   (MGI Ref ID J:197168)
  • muscle phenotype
  • muscle spasm   (MGI Ref ID J:197168)
View Research Applications

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

Neurobiology Research
Ataxia (Movement) Defects
Behavioral and Learning Defects
Epilepsy

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Tg(Lmnb1)1Yfu
Allele Name transgene insertion 1, Ying-Hui Fu
Allele Type Transgenic (Inserted expressed sequence)
Common Name(s) Lmnb1BAC;
Strain of OriginC57BL/6J
Expressed Gene Lmnb1, lamin B1, mouse, laboratory
Promoter Lmnb1, lamin B1, mouse, laboratory
Molecular Note The transgene contains BAC RP23-460J18. Lines 1 and 2 were generated with 4- and 2.5-fold increased expression, respectively. [MGI Ref ID J:197168]
 

Genotyping

Genotyping Information

Genotyping Protocols

Tg(LMNB1)1Yfu-5', Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Additional References

Tg(Lmnb1)1Yfu related

Heng MY; Lin ST; Verret L; Huang Y; Kamiya S; Padiath QS; Tong Y; Palop JJ; Huang EJ; Ptacek LJ; Fu YH. 2013. Lamin B1 mediates cell-autonomous neuropathology in a leukodystrophy mouse model. J Clin Invest 123(6):2719-29. [PubMed: 23676464]  [MGI Ref ID J:197168]

Health & husbandry

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.

Health & Colony Maintenance Information

Animal Health Reports

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

Colony Maintenance

Breeding & HusbandryHemizygotes are viable and fertile, but homozygotes are not.

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* $2140.00
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* $2782.00
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
   Wild-type 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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