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- Description
- Disease & phenotype
- Genes & alleles
- Genotyping
- Health & care
- References
- Pricing & purchasing
- Terms of use
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 B6.129-Kns2tm1Gsn/J (Changed: 12-MAR-07 ) B6.129-Klc1tm1Gsn (Changed: 15-DEC-04 ) Type Congenic; Mutant Strain; Targeted Mutation; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Additional information on Congenic nomenclature. Species laboratory mouse Generation N9
Generation DefinitionsDonating Investigator Dr. Lawrence S.B. Goldstein, UCSD-HHMI Description
The donating investigator indicates that mice that are homozygous for the targeted allele on a C57BL/6 genetic background exhibit significant perinatal mortality (60%). Mortality seems not to be a factor on a mixed B6;129 background. Surviving mice are noticeably smaller than wildtype mice. Mice surviving to adulthood breed poorly, possibly due to less than adequate nurturing capabilities. Minor amounts of a functionless, truncated protein product can be detected. Motor defects are evident, as are alterations in intracellular localization of kinesin-I and COP-I components.Development
A targeting vector containing an IRES/beta-geo cassette was used to disrupt a portion of the targeted gene encoding the conserved TPR region. The construct was electroporated into 129X1/SvJ x 129S1/Sv-derived R1 embryonic stem (ES) cells. Correctly targeted ES cells were injected into C57BL/6J blastocysts. The resulting chimeric animals were crossed to C57BL/6 mice for at least six generations (6/01).
Related Strains
Alzheimer's Disease Models
View Alzheimer's Disease Models (108 strains)
008300 B6.Cg-Tg(CMV-Klc1)73Gsn/J 008301 B6.Cg-Tg(CMV-Klc1)90Gsn/J
Additional Web Information
Visit the Alzheimer's Disease Mouse Model Resource site for helpful information on Alzheimer's Disease and research resources.
Phenotype
Phenotype Information
assigned by genotype
Klc1tm1Gsn/Klc1tm1Gsn
B6.129-Klc1tm1Gsn
- nervous system phenotype
- abnormal axon morphology
- proximal axonal swelling is visible in cervical spinal cord, swelling is progressive and age-dependent (MGI Ref ID J:148380)
- axonal swelling observed in ventral roots of spinal cord (MGI Ref ID J:148380)
- the number of large caliber axons are reduced in peripheral motor roots (MGI Ref ID J:148380)
- swollen and distended axons with disrupted microtubule networks, anomalous tubuloreticular structures and atypical accumulations of membrane stacks are observed in hippocampal neurons (MGI Ref ID J:148380)
- abnormal axonal transport
- polarized neurons transiently transfected with a YFP-APP fusion exhibit a decrease in the proportion of amyloid precursor protein (APP) vesicles moving in the anterograde direction, an increase in retrograde vesicles and a reduction in net velocity and average run length for anterograde and retrograde vesicles (MGI Ref ID J:148380)
- phosphorylated neurofilaments are increased in the dentate gyrus and CA1 region in 18 month old mice (MGI Ref ID J:148380)
- abnormal brain white matter morphology
- reduction of white matter is observed in corpus callosum (MGI Ref ID J:148380)
- abnormal spinal cord white matter morphology
- reduction of white matter is observed in the cervical spinal cord (MGI Ref ID J:148380)
- axon degeneration
- degeneration is observed in the corpus callosum, anterior commissure of the brain in 18 month old mice (MGI Ref ID J:148380)
- tau protein deposits
- phosphorylated tau deposits are observed in ventral axons along roots of the spinal cord, however, no abnormal accumulations are observed in neuronal cell bodies (MGI Ref ID J:148380)
- soluble levels of total and phosphorylated tau are increased in brainstem and spinal cord, and insoluble tau forms are increased in spinal cord, however, overall levels are similar to wild-type (MGI Ref ID J:148380)
The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.
Klc1tm1Gsn/Klc1tm1Gsn
either: (involves: 129S/SvEv * 129S1/Sv * 129X1/SvJ) or (involves: 129S1/Sv * 129X1/SvJ * C57BL/6)
- growth/size phenotype
- decreased body size
- significantly smaller than normal (MGI Ref ID J:57877)
- behavior/neurological phenotype
- impaired coordination
- reduced ability to hang upside down from chicken wire (MGI Ref ID J:57877)
Klc1tm1Gsn/Klc1tm1Gsn
involves: 129S1/Sv * 129X1/SvJ
- cellular phenotype
- abnormal vesicle-mediated transport
- neurons exhibit impaired anterograde and retrograde vesicle transport compared with wild-type cells (MGI Ref ID J:170887)
This mouse can be used to support research in many areas including:Klc1tm1Gsn related
Developmental Biology Research
Growth Defects
Neurobiology Research
Alzheimer's Disease
Ataxia (Movement) Defects
Neurodegeneration
Genes & Alleles
Gene & Allele Information provided by MGI
| Allele Symbol | Klc1tm1Gsn | ||
|---|---|---|---|
| Allele Name | targeted mutation 1, Lawrence S B Goldstein | ||
| Allele Type | Targeted (Reporter) | ||
| Mutation Made By | Elizabeth Roberts, University of California, San Diego | ||
| Strain of Origin | (129X1/SvJ x 129S1/Sv)F1-Kitl<+> | ||
| ES Cell Line Name | R1 | ||
| ES Cell Line Strain | (129X1/SvJ x 129S1/Sv)F1-Kitl<+> | ||
| Gene Symbol and Name | Klc1, kinesin light chain 1 | ||
| Chromosome | 12 | ||
| Gene Common Name(s) | AI874768; KLC; KNS2; KNS2A; Kns2; expressed sequence AI874768; kinesin 2; | ||
| Molecular Note | An IRES Beta-geo cassette replaced the exon that encodes the entire first TPR domain and part of the second TPR domain. Western analysis of crude cytoplasmic brain extracts of homozygous mutant mice detected dramatically reduced levels of full length KLC1, as well as very small amounts of truncated KLC1. Immunoprecipitation studies did not detect either form of KLC1 in KIF5A or KIF5B coprecipitates of brain extracts of homozygous mutant mice. Immunofluorescence studies of sensory and motor neuron cell bodies in dorsal root ganglion and spinal cord of homozygous mutant mice detected very faint KCL1 staining. [MGI Ref ID J:57877] | ||
Genotyping
Genotyping Information
Genotyping Protocols
Klc1tm1Gsn, Standard PCR
Helpful Links
Genotyping resources and troubleshooting
References
References provided by MGI
Selected Reference(s)
Rahman A; Kamal A; Roberts EA; Goldstein LS. 1999. Defective kinesin heavy chain behavior in mouse kinesin light chain mutants. J Cell Biol 146(6):1277-88. [PubMed: 10491391] [MGI Ref ID J:57877]
Additional References
Klc1tm1Gsn relatedEncalada SE; Szpankowski L; Xia CH; Goldstein LS. 2011. Stable kinesin and dynein assemblies drive the axonal transport of mammalian prion protein vesicles. Cell 144(4):551-65. [PubMed: 21335237] [MGI Ref ID J:170887]
Falzone TL; Gunawardena S; McCleary D; Reis GF; Goldstein LS. 2010. Kinesin-1 transport reductions enhance human tau hyperphosphorylation, aggregation and neurodegeneration in animal models of tauopathies. Hum Mol Genet 19(22):4399-408. [PubMed: 20817925] [MGI Ref ID J:165140]
Falzone TL; Stokin GB; Lillo C; Rodrigues EM; Westerman EL; Williams DS; Goldstein LS. 2009. Axonal stress kinase activation and tau misbehavior induced by kinesin-1 transport defects. J Neurosci 29(18):5758-67. [PubMed: 19420244] [MGI Ref ID J:148380]
Stokin GB; Lillo C; Falzone TL; Brusch RG; Rockenstein E; Mount SL; Raman R; Davies P; Masliah E; Williams DS; Goldstein LS. 2005. Axonopathy and transport deficits early in the pathogenesis of Alzheimer's disease. Science 307(5713):1282-8. [PubMed: 15731448] [MGI Ref ID J:96346]
Szpankowski L; Encalada SE; Goldstein LS. 2012. Subpixel colocalization reveals amyloid precursor protein-dependent kinesin-1 and dynein association with axonal vesicles. Proc Natl Acad Sci U S A 109(22):8582-7. [PubMed: 22582169] [MGI Ref ID J:184761]
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 & Husbandry When maintaining a live colony, these mice are maintained as heterozygotes.
Pricing and Purchasing
Pricing, Supply Level & Notes, Controls
| Pricing for USA, Canada and Mexico shipping destinations |
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Cryopreserved
Cryopreserved Mice - Ready for Recovery
Animals Provided
Price (US dollars $) Cryorecovery* $3000.00 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 |
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Cryopreserved
Cryopreserved Mice - Ready for Recovery
Animals Provided
Price (US dollars $) Cryorecovery* $3900.00 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).
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Standard Supply
Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.
General Supply Notes
- Genomic DNA is available for this strain from the Mouse DNA Resource.
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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.Ordering Information
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