Strain Name:

B6.Cg-Aarssti/J

Stock Number:

002560

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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 B6.Cg-sti/J    (Changed: 15-AUG-06 )
B6.STOCK-sti/J    (Changed: 15-AUG-06 )
B6.STOCK-sti    (Changed: 15-AUG-06 )
Type Congenic; Mutant Strain;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Specieslaboratory mouse
H2 Haplotypeb

Appearance
black
Related Genotype: a/a

Description
Mice homozygous for this spontaneous mutation exhibit a rough, sticky coat, progressive ataxia and Purkinje cell degeneration. Cerebellar Purkinje cell loss is first observed by three weeks of age becomes extensive by six weeks of age and continues to progress slowly over the course of a year. This mutant mouse strain may be useful in studies related to neurodegeneration, protein misfolding and aberrant transfer RNAs.

Development
This spontaneous mutation arose in a mixed background stock, which included the wild-derived PERU. The colony was transferred from the laboratory of Margaret Wallace at the University of Cambridge to The Jackson Laboratory in approximately 1984 and was backcrossed to C57BL/6J for 9 generations. Embryos were cryopreserved in 1996 using N9F1 heterozygous males bred to C57BL/6J females.

Additional Web Information

JAX® NOTES, Fall 2006; 503. "Sticky" Mice Lead to Discovery of New Cause of Neurodegenerative Disease.

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.
Charcot-Marie-Tooth Disease, Axonal, Type 2n; CMT2N   (AARS)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Aarssti/Aarssti

        B6.Cg-Aarssti
  • nervous system phenotype
  • Purkinje cell degeneration
    • loss of cells beginning at three weeks of age   (MGI Ref ID J:110647)
    • extensive cell loss by six weeks of age especially in rostral cerebellum   (MGI Ref ID J:110647)
  • behavior/neurological phenotype
  • ataxia
    • progressive ataxia   (MGI Ref ID J:110647)
  • impaired balance
    • as demonstrated by rotorod   (MGI Ref ID J:110647)
  • tremors
    • mild tremors occur at 6 weeks of age and progress to ataxia   (MGI Ref ID J:110647)
  • integument phenotype
  • abnormal hair shaft morphology
    • hair fibers break at follicular ostium and have cuff of compacted inner root sheath cells   (MGI Ref ID J:110647)
  • disheveled coat
    • fur has rough, unkempt, sticky appearance   (MGI Ref ID J:110647)
    • electron microscopy indicates short, broken, deformed hair fibers   (MGI Ref ID J:110647)
  • focal hair loss
    • patchy hair loss occurs over time   (MGI Ref ID J:110647)
  • hair follicle degeneration
    • hair fibers twisted within the follicle   (MGI Ref ID J:110647)
  • hair follicle outer rooth sheath hyperplasia
    • hyperplasia of outer root sheath   (MGI Ref ID J:110647)
View Research Applications

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

Aarssti related

Dermatology Research
Skin and Hair Texture Defects

Neurobiology Research
Ataxia (Movement) Defects
Cerebellar Defects
      Purkinje cell defect
Neurodegeneration

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Aarssti
Allele Name sticky
Allele Type Spontaneous
Strain of OriginLaboratory stock
Gene Symbol and Name Aars, alanyl-tRNA synthetase
Chromosome 8
Gene Common Name(s) AI316495; C76919; CMT2N; MGC:37368; expressed sequence AI316495; expressed sequence C76919; sti; sticky;
General Note The sticky mutation arose on an unknown mouse stock.
Molecular Note A C-to-A nucelotide change at nucleotide 2201 is predicted to result in an alanine to glutamic acid non-conservative amino acid change in codon 774 of the encoded protein. Transgenic rescue experiments using a wild-type Aars gene indicated that this transversion mutation is responsible for the mutant phenotype. [MGI Ref ID J:110647]

Genotyping

Genotyping Information


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Lee JW; Beebe K; Nagle LA; Jang J; Longo-Guess CM; Cook SA; Davisson MT; Sundberg JP; Schimmel P; Ackerman SL. 2006. Editing-defective tRNA synthetase causes protein misfolding and neurodegeneration Nature 443(7107):50-5. [PubMed: 16906134]  [MGI Ref ID J:110647]

Additional References

Aarssti related

Sarna JR; Hawkes R. 2011. Patterned Purkinje cell loss in the ataxic sticky mouse. Eur J Neurosci 34(1):79-86. [PubMed: 21645134]  [MGI Ref ID J:177943]

Stum M; McLaughlin HM; Kleinbrink EL; Miers KE; Ackerman SL; Seburn KL; Antonellis A; Burgess RW. 2011. An assessment of mechanisms underlying peripheral axonal degeneration caused by aminoacyl-tRNA synthetase mutations. Mol Cell Neurosci 46(2):432-43. [PubMed: 21115117]  [MGI Ref ID J:171297]

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* $3175.00
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 11 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* $4127.50
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 11 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.

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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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