Description

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 Mutant Mice. Species laboratory mouse H2 Haplotype b

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

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

Phenotype

Phenotype Information

View Mammalian Phenotype Terms

Mammalian Phenotype Terms

      assigned by genotype

Aars^sti/Aars^sti

        B6.Cg-Aars^sti

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

View Research Applications

Research Applications

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

Aars^sti related

Dermatology Research
Skin and Hair Texture Defects

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

Genes & Alleles

Gene & Allele Information

Allele Symbol		Aarssti
Allele Name		sticky
Allele Type		Spontaneous
Strain of Origin		Laboratory stock
Gene Symbol and Name		Aars, alanyl-tRNA synthetase
Chromosome		8
Gene Common Name(s)		AI316495; C76919; 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

This strain will not have a genotyping protocol or one is not currently available.

Helpful Links

Optimizing PCR Protocols

References

References

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]

Health & husbandry

Health & Colony Maintenance Information

Currently there no information available for this strain. This may be due to the supply level of this strain.

Purchasing information

Pricing, Supply Level & Notes, Controls, General Terms & Conditions

Pricing

Supply Details

Standard Supply

Repository-Cryopreserved. Must Be Recovered. Please refer to pricing and supply notes for further information.

Supply Notes

Cryorecovery - Standard. The recovery process begins when a signed agreement form is returned to the Customer Service Department after order placement. Although results vary by strain, at least two males and two females (two pairs) will be provided, typically within 15 weeks of our receipt of the signed agreement form. If the first recovery attempt is unsuccessful or only one pair is recovered, a second recovery will be done, extending the delivery time to approximately 25 weeks. At least one member of each pair will be of known genotype and will carry the mutation if it is a mutant strain. Please note that 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. Price represents a repository maintenance fee, which includes the cost of recovery of the strain from the cryopreservation resource and the periodic replacement of the frozen embryos used for recovery. Cryorecovery to establish a Dedicated Supply for greater quantities of mice. One to two pairs will be recovered to establish a Dedicated Supply of mice. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 or 1-207-288-5845.

General Terms and Conditions

See Terms of Use

The Jackson Laboratory's Genotype Promise

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.

---

Ordering and Purchasing Information

      Purchasing Information
      JAX^® Mice Orders
      Surgical Services

Contact Information
Orders & Technical Support
Tel: 800.422.6423 or 207.288.5845
Fax: 207.288.6150
Technical Support Email Form

Terms of Use

Terms of Use

General Terms and Conditions

Contact information

General inquiries

Contracts Administration

phone:	207-288-6470
fax:	207-288-6655

JAX^® Mice & Services Conditions of Use

“Each recipient institution, including its employees and other researchers under its control (RECIPIENT), of mice or services using mice from The Jackson Laboratory (TJL) agrees that such mice, descendants of those mice derived by inbreeding or crossbreeding, including unmodified derivatives of those mice or their descendants (“MICE”) shall not be: (i) used for any purpose other than the internal research of the RECIPIENT, (ii) sold or otherwise provided to any third party for any use, or (iii) provided to any agent or other third party to provide breeding or other services with respect to MICE. Acceptance of MICE from TJL shall be deemed agreement by RECIPIENT to these conditions, and departure from these conditions requires The Jackson Laboratory’s prior written authorization.”

In case of dissatisfaction for a valid reason and claimed in writing by a purchaser within ninety (90) days of receipt of MICE, products or services, The Jackson Laboratory will, at its option, provide credit or replacement for the MICE or product received or the services provided.

No Liability

In no event shall The Jackson Laboratory, its trustees, directors, officers, employees, and affiliates be liable for any causes of action or damages, including any direct, indirect, special, or consequential damages, arising out of the provision of MICE, products or services, including economic damage or injury to property and lost profits, and including any damage arising from acts or negligence on the part of The Jackson Laboratory, its agents or employees. In purchasing or receiving MICE, products or services from The Jackson Laboratory, purchaser or recipient, or any party claiming by or through them, expressly releases and discharges The Jackson Laboratory from all such causes of action or damages, and further agrees to defend and indemnify The Jackson Laboratory from any costs or damages arising out of any third party claims.

MICE and biological materials are to be used in a safe manner and in accordance with all applicable governmental rules and regulations.

The foregoing represents the General Terms and Conditions applicable to The Jackson Laboratory’s MICE, products and services. In addition, special terms and conditions of sale of certain MICE, products and services may be set forth separately in The Jackson Laboratory web pages, catalogs, price lists, contracts, and/or other documents, and these special terms and conditions shall also govern the sale of these MICE, products and services by The Jackson Laboratory, and by its licensees and distributors.

Acceptance of delivery of MICE, products or services shall be deemed agreement to these terms and conditions. No purchase order or other document transmitted by purchaser or recipient that may modify the terms and conditions hereof, shall be in any way binding on The Jackson Laboratory, and instead the terms and conditions set forth herein, including any special terms and conditions set forth separately, shall govern the sale of MICE, products services by The Jackson Laboratory.