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 Donating Investigator Dr. Lawrence S.B. Goldstein, UCSD-HHMI
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.
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).
Alzheimer's Disease Models
005987 129-Achetm1Loc/J 006409 129S1.129(Cg)-Tg(APPSw)40Btla/Mmjax 008077 129S1/Sv-Bchetm1Loc/J 016198 129S6.Cg-Tg(Camk2a-tTA)1Mmay/JlwsJ 014556 129S6/SvEv-Apoetm4Mae/J 006555 A.129(B6)-Tg(APPSw)40Btla/Mmjax 005708 B6.129-Apbb1tm1Quhu/J 004714 B6.129-Bace1tm1Pcw/J 004193 B6.129-Psen1tm1Mpm/J 003615 B6.129-Psen1tm1Shn/J 005300 B6.129-Tg(APPSw)40Btla/Mmjax 005617 B6.129P-Psen2tm1Bdes/J 002609 B6.129P2-Nos2tm1Lau/J 007685 B6.129P2-Psen1tm1Vln/J 007999 B6.129P2-Sorl1Gt(Ex255)Byg/J 008087 B6.129S1-Bchetm1Loc/J 002509 B6.129S2-Plautm1Mlg/J 005301 B6.129S2-Tg(APP)8.9Btla/J 004163 B6.129S4-Cdk5r1tm1Lht/J 010959 B6.129S4-Grk5tm1Rjl/J 010960 B6.129S4-Grk5tm2Rjl/J 002213 B6.129S4-Ngfrtm1Jae/J 006406 B6.129S4-Tg(APPSwLon)96Btla/Mmjax 006469 B6.129S4-Tg(PSEN1H163R)G9Btla/J 012564 B6.129S5-Dhcr24tm1Lex/SbpaJ 004142 B6.129S7-Aplp2tm1Dbo/J 004133 B6.129S7-Apptm1Dbo/J 007251 B6.129X1-Mapttm1Hnd/J 013040 B6.Cg-Apoetm1Unc Ins2Akita/J 005642 B6.Cg-Clutm1Jakh/J 005491 B6.Cg-Mapttm1(EGFP)Klt Tg(MAPT)8cPdav/J 009126 B6.Cg-Nos2tm1Lau Tg(Thy1-APPSwDutIowa)BWevn/Mmjax 005866 B6.Cg-Tg(APP695)3Dbo Tg(PSEN1dE9)S9Dbo/Mmjax 008730 B6.Cg-Tg(APPSwFlLon,PSEN1*M146L*L286V)6799Vas/Mmjax 005864 B6.Cg-Tg(APPswe,PSEN1dE9)85Dbo/Mmjax 007575 B6.Cg-Tg(CAG-Ngb,-EGFP)1Dgrn/J 016197 B6.Cg-Tg(CAG-OTC/CAT)4033Prab/J 005855 B6.Cg-Tg(Camk2a-Prkaca)426Tabe/J 007004 B6.Cg-Tg(Camk2a-tTA)1Mmay/DboJ 004996 B6.Cg-Tg(DBH-Gal)1923Stei/J 007673 B6.Cg-Tg(Gad1-EGFP)3Gfng/J 004662 B6.Cg-Tg(PDGFB-APP)5Lms/J 006293 B6.Cg-Tg(PDGFB-APPSwInd)20Lms/2Mmjax 006006 B6.Cg-Tg(Prnp-APP)A-2Dbo/J 008596 B6.Cg-Tg(Prnp-Abca1)EHol/J 006005 B6.Cg-Tg(Prnp-App/APPswe)E1-2Dbo/Mmjax 007180 B6.Cg-Tg(Prnp-ITM2B/APP695*40)1Emcg/J 007182 B6.Cg-Tg(Prnp-ITM2B/APP695*42)A12Emcg/J 005999 B6.Cg-Tg(SBE/TK-luc)7Twc/J 012597 B6.Cg-Tg(Thy1-COL25A1)861Yfu/J 007051 B6.Cg-Tg(tetO-APPSwInd)102Dbo/Mmjax 007052 B6.Cg-Tg(tetO-APPSwInd)107Dbo/Mmjax 007049 B6.Cg-Tg(tetO-APPSwInd)885Dbo/Mmjax 009337 B6.FVB-Tg(Prnp-RTN3)2Yanr/J 006394 B6;129-Apba2tm1Sud Apba3tm1Sud Apba1tm1Sud/J 008364 B6;129-Chattm1(cre/ERT)Nat/J 008476 B6;129-Ncstntm1Sud/J 004807 B6;129-Psen1tm1Mpm Tg(APPSwe,tauP301L)1Lfa/Mmjax 007605 B6;129P-Psen1tm1Vln/J 005618 B6;129P2-Bace2tm1Bdes/J 008333 B6;129P2-Dldtm1Ptl/J 002596 B6;129P2-Nos2tm1Lau/J 003822 B6;129S-Psen1tm1Shn/J 012639 B6;129S4-Mapttm3(HDAC2)Jae/J 012869 B6;129S6-Apbb2tm1Her/J 006410 B6;129S6-Chattm2(cre)Lowl/J 005993 B6;129S6-Pcsk9tm1Jdh/J 008636 B6;C-Tg(Prnp-APP695*/EYFP)49Gsn/J 007002 B6;C3-Tg(Prnp-ITM2B/APP695*42)A12Emcg/Mmjax 008169 B6;C3-Tg(Prnp-MAPT*P301S)PS19Vle/J 000231 B6;C3Fe a/a-Csf1op/J 008850 B6;SJL-Tg(Mt1-LDLR)93-4Reh/AgnJ 003378 B6C3-Tg(APP695)3Dbo Tg(PSEN1)5Dbo/J 004462 B6C3-Tg(APPswe,PSEN1dE9)85Dbo/Mmjax 003741 B6D2-Tg(Prnp-MAPT)43Vle/J 016556 B6N.129-Ptpn5tm1Pjlo/J 018957 B6N.129S6(B6)-Chattm2(cre)Lowl/J 024841 B6N.Cg-Tg(Prnp-MAPT*P301S)PS19Vle/J 006554 B6SJL-Tg(APPSwFlLon,PSEN1*M146L*L286V)6799Vas/Mmjax 012621 C.129S(B6)-Chrna3tm1.1Hwrt/J 002328 C.129S2-Plautm1Mlg/J 003375 C3B6-Tg(APP695)3Dbo/Mmjax 005087 C57BL/6-Tg(Camk2a-IDE)1Selk/J 005086 C57BL/6-Tg(Camk2a-MME)3Selk/J 008833 C57BL/6-Tg(Camk2a-UBB)3413-1Fwvl/J 007027 C57BL/6-Tg(Thy1-APPSwDutIowa)BWevn/Mmjax 010800 C57BL/6-Tg(Thy1-PTGS2)300Kand/J 010703 C57BL/6-Tg(Thy1-PTGS2)303Kand/J 005706 C57BL/6-Tg(tetO-CDK5R1/GFP)337Lht/J 006618 C57BL/6-Tg(tetO-COX8A/EYFP)1Ksn/J 007677 CB6-Tg(Gad1-EGFP)G42Zjh/J 007072 CByJ.129P2(B6)-Nos2tm1Lau/J 006472 D2.129(B6)-Tg(APPSw)40Btla/Mmjax 007067 D2.129P2(B6)-Apoetm1Unc/J 013719 D2.Cg-Apoetm1Unc Ins2Akita/J 003718 FVB-Tg(GadGFP)45704Swn/J 013732 FVB-Tg(NPEPPS)1Skar/J 013156 FVB-Tg(tetO-CDK5R1*)1Vln/J 015815 FVB-Tg(tetO-MAPT*P301L)#Kha/JlwsJ 002329 FVB.129S2-Plautm1Mlg/J 003753 FVB/N-Tg(Eno2CDK5R1)1Jdm/J 006143 FVB/N-Tg(Thy1-cre)1Vln/J 008051 NOD.129P2(B6)-Ctsbtm1Jde/RclJ 008390 STOCK Apptm1Sud/J 012640 STOCK Hdac2tm1.2Rdp/J 004808 STOCK Mapttm1(EGFP)Klt Tg(MAPT)8cPdav/J 004779 STOCK Mapttm1(EGFP)Klt/J 014092 STOCK Tg(ACTB-tTA2,-MAPT/lacZ)1Luo/J 014544 STOCK Tg(tetO-ABL1*P242E*P249E)CPdav/JView Alzheimer's Disease Models (109 strains)Strains carrying other alleles of Klc1
008300 B6.Cg-Tg(CMV-Klc1)73Gsn/J 008301 B6.Cg-Tg(CMV-Klc1)90Gsn/JView Strains carrying other alleles of Klc1 (2 strains)
View Mammalian Phenotype TermsMammalian Phenotype Terms provided by MGIassigned by genotype
- 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 is associated with a similar, but not exact match to this JAX® Mice strain.
Klc1tm1Gsn/Klc1tm1Gsneither: (involves: 129S/SvEv * 129S1/Sv * 129X1/SvJ) or (involves: 129S1/Sv * 129X1/SvJ * C57BL/6)
- growth/size/body 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/Klc1tm1Gsninvolves: 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)View Research Applications
|Allele Name||targeted mutation 1, Lawrence S B Goldstein|
|Allele Type||Targeted (Null/Knockout, 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|
|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]|
Encalada 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]
Animal Health ReportsProduction of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200.
Breeding & Husbandry When maintaining a live colony, these mice are maintained as heterozygotes.
|Pricing for USA, Canada and Mexico shipping destinations|
Cryopreserved Mice - Ready for Recovery
Price (US dollars $) Cryorecovery* $3300.00
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.
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|
Cryopreserved Mice - Ready for Recovery
Price (US dollars $) Cryorecovery* $4290.00
Cryorecovery - Standard.
Progeny testing is not required.
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