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Former Names B6;129S-Fcgr2btm1Rav Clcn1adr-mto5J/J (Changed: 03-AUG-07 ) Type Mutant Strain; Targeted Mutation; Additional information on Genetically Engineered Mutant Mice. Species laboratory mouse Generation +N1 Description
B6;129S-Fcgr2btm1Ttk Clcn1adr-mto5J mice homozygous for Clcn1adr-mto5J are affected by a neuromuscular disorder that manifests by weaning as an apparent weakness or stiffness in the rear legs, which is particularly noticeable when the mice are picked up and put down again (as when being transferred to a new cage). The phenotype may disappear as the mice get older. Both male and female homozygotes have normal lifespans and are fertile.Development
The Clcn1adr-mto5J mutation occurred spontaneously in the B6;129S-Fcgr2btm1Ttk/J (002848) colony at The Jackson Laboratory in October, 1999. It was shown to be recessive by the lack of a phenotype in F1 progeny of crosses to C57BL/6J, and it was shown to be a remutation at Clcn1 by its failure to complement Clcn1adr-mto2J in an allele test.
| The most appropriate controls for the Clcn1adr-mto5J mutant mice are littermates either wildtype or heterozygous for Clcn1adr-mto5J (and, ideally, matched for their Fcgr2btm1Rav genotype). Alternatively, B6129SF2 mice (Stock No. 101045) may be used as controls; the latter provide only an approximate genetic match to the mixed B6;129 background and lack the Fcgr2btm1Rav. | ||
| Considerations for Choosing Controls | ||
Strains carrying Fcgr2btm1Ttk allele
002848 B6;129S4-Fcgr2btm1Ttk/J View Strains carrying Fcgr2btm1Ttk (1 strain)
Strains carrying other alleles of Clcn1
003922 BALB/cByJ-Clcn1adr-mto2J jgl/J 001265 BALB/cByJ-Clcn1adr-mto2J/J 005253 C57BL/6J-Clcn1adr-mto6J/J 005465 C57BL/6J-Clcn1adr-mto7J/J 008253 C57BL/6J-Clcn1adr-mto9J/J 000939 SWR/J-Clcn1adr-mto/J View Strains carrying other alleles of Clcn1 (6 strains)
View Mammalian Phenotype Terms
Mammalian Phenotype Terms
assigned by genotype
Clcn1adr-mto5J/Clcn1adr-mto5J
B6;129S-Fcgr2btm1Rav
- behavior/neurological phenotype
- abnormal stationary movement (MGI Ref ID J:94340)
- beginning at weaning, mice have weakness or stiffness in hindlimbs
- however, defect may disappear with age
- weakness (MGI Ref ID J:94340)
- beginning at weaning, mice have weakness or stiffness in hindlimbs
- however, defect may disappear with age
View Research Applications
Research Applications
This mouse can be used to support research in many areas including:Clcn1adr-mto5J related
Fcgr2btm1Ttk relatedCell Biology Research
Channel and Transporter Defects (chloride)
Mouse/Human Gene Homologs
myotonia congenita, autosomal dominant, Thomsen disease)
Neurobiology Research
Channel and Transporter Defects (chloride)
Neuromuscular Defects
Immunology and Inflammation Research
CD Antigens, Antigen Receptors, and Histocompatibility Markers
Inflammation
| Allele Symbol | Clcn1adr-mto5J | ||
|---|---|---|---|
| Allele Name | myotonia 5 Jackson | ||
| Allele Type | Spontaneous | ||
| Strain of Origin | B6;129S-Fcgr2btm1Rav | ||
| Gene Symbol and Name | Clcn1, chloride channel 1 | ||
| Chromosome | 6 | ||
| Gene Common Name(s) | CLC1; Clc-1; Clc1; MGC138361; MGC142055; NMF355; SMCC; SMCC1; adr; arrested development of righting response; mto; myotonia; neuroscience mutagenesis facility, 355; nmf310; nmf355; | ||
| General Note | Mice homozygous for Clcn1adr-mto5J are affected by a neuromuscular disorder that manifests by weaning as an apparent weakness or stiffness in the rear legs, which is particularly noticeable when the mice are picked up and put down again (as when being transferred to a new cage). The phenotype may disappear as the mice get older. Both male and female homozygotes have normal lifespans and are fertile. | ||
| Molecular Note | This mutation occurred spontaneously in a colony at The Jackson Laboratory in October, 1999. It was shown to be a remutation at Clcn1 by its failure to complement Clcn1adr-mto2J in a complementation test. | ||
| Allele Symbol | Fcgr2btm1Ttk | ||
| Allele Name | targeted mutation 1, Toshiyuki Takai | ||
| Allele Type | Targeted (knock-out) | ||
| Common Name(s) | FcgammaRII; FcgammaRII-; FcgammaRII2B-; FcgammaRIIB-; Fcgr2btm1Rav; IIB-; | ||
| Mutation Made By | Jeffrey Ravetch, Memorial Sloan Kettering Cancer Center | ||
| Strain of Origin | 129S4/SvJae | ||
| ES Cell Line Name | J1 | ||
| ES Cell Line Strain | 129S4/SvJae | ||
| Gene Symbol and Name | Fcgr2b, Fc receptor, IgG, low affinity IIb | ||
| Chromosome | 1 | ||
| Gene Common Name(s) | AI528646; CD32; CD32B; F630109E10Rik; FCG2; FCGR2; FCRII; Fc gamma RIIB; Fc receptor, IgG, low affinity II; Fc receptor, IgG, low affinity IIa; Fc-gamma receptor 2; Fc-gamma receptor 3; Fc[g]RII; FcgRII; FcgammaRIIB; Fcgr2; Fcgr2a; Fcr-2; Fcr-3; IGFR2; Ly-17; Ly-m20; LyM-1; RIKEN cDNA F630109E10 gene; expressed sequence AI528646; lymphocyte antigen 17; lymphocyte antigen m20; | ||
| Molecular Note | A 2.3 kb genomic fragment containing the S1 and EC1 exons was replaced by a neomycin resistance cassette. Encoded transcript was undetectable in homozygous mutant mice via Northern blot analysis (data not shown). Western blot analysis of LPS-stimulatedB cells derived from homozygous mice confirmed that no protein was made from this allele, and FACS analysis demonstrated that no functional protein was expressed on the cell surface of B cells in homozygous mice. [MGI Ref ID J:31133] | ||
Genotyping Protocols
Fcgr2btm1Ttk, STD PCR, vers. 1
Helpful Links
Optimizing PCR Protocols
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Feng HM; Walker DH. 2004. Mechanisms of immunity to Ehrlichia muris: a model of monocytotropic ehrlichiosis. Infect Immun 72(2):966-71. [PubMed: 14742542] [MGI Ref ID J:87862]
Fukuyama H; Nimmerjahn F; Ravetch JV. 2005. The inhibitory Fcgamma receptor modulates autoimmunity by limiting the accumulation of immunoglobulin G+ anti-DNA plasma cells. Nat Immunol 6(1):99-106. [PubMed: 15592473] [MGI Ref ID J:94871]
Getahun A; Dahlstrom J; Wernersson S; Heyman B. 2004. IgG2a-mediated enhancement of antibody and T cell responses and its relation to inhibitory and activating Fc gamma receptors. J Immunol 172(9):5269-76. [PubMed: 15100265] [MGI Ref ID J:89643]
Gjertsson I; Kleinau S; Tarkowski A. 2002. The impact of Fcgamma receptors on Staphylococcus aureus infection. Microb Pathog 33(4):145-52. [PubMed: 12385742] [MGI Ref ID J:102982]
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Hamaguchi Y; Xiu Y; Komura K; Nimmerjahn F; Tedder TF. 2006. Antibody isotype-specific engagement of Fcgamma receptors regulates B lymphocyte depletion during CD20 immunotherapy. J Exp Med 203(3):743-53. [PubMed: 16520392] [MGI Ref ID J:123761]
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Mold C; Rodic-Polic B; Du Clos TW. 2002. Protection from Streptococcus pneumoniae infection by C-reactive protein and natural antibody requires complement but not Fc gamma receptors. J Immunol 168(12):6375-81. [PubMed: 12055255] [MGI Ref ID J:123793]
Mold C; Rodriguez W; Rodic-Polic B; Du Clos TW. 2002. C-reactive protein mediates protection from lipopolysaccharide through interactions with Fc gamma R. J Immunol 169(12):7019-25. [PubMed: 12471137] [MGI Ref ID J:118418]
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Muhlfeld AS; Segerer S; Hudkins K; Carling MD; Wen M; Farr AG; Ravetch JV; Alpers CE. 2003. Deletion of the fcgamma receptor IIb in thymic stromal lymphopoietin transgenic mice aggravates membranoproliferative glomerulonephritis. Am J Pathol 163(3):1127-36. [PubMed: 12937154] [MGI Ref ID J:85173]
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Nakamura A; Yuasa T; Ujike A; Ono M; Nukiwa T; Ravetch JV; Takai T. 2000. Fcgamma receptor IIB-deficient mice develop Goodpasture's syndrome upon immunization with type IV collagen: a novel murine model for autoimmune glomerular basement membrane disease. J Exp Med 191(5):899-906. [PubMed: 10704470] [MGI Ref ID J:60920]
Nakamura K; Hirai H; Torashima T; Miyazaki T; Tsurui H; Xiu Y; Ohtsuji M; Lin QS; Tsukamoto K; Nishimura H; Ono M; Watanabe M; Hirose S. 2007. CD3 and immunoglobulin G Fc receptor regulate cerebellar functions. Mol Cell Biol 27(14):5128-34. [PubMed: 17502348] [MGI Ref ID J:123637]
Nakamura K; Malykhin A; Coggeshall KM. 2002. The Src homology 2 domain-containing inositol 5-phosphatase negatively regulates Fcgamma receptor-mediated phagocytosis through immunoreceptor tyrosine-based activation motif-bearing phagocytic receptors. Blood 100(9):3374-82. [PubMed: 12384440] [MGI Ref ID J:115560]
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Nishimura H; Strominger JL. 2006. Involvement of a tissue-specific autoantibody in skin disorders of murine systemic lupus erythematosus and autoinflammatory diseases. Proc Natl Acad Sci U S A 103(9):3292-7. [PubMed: 16492738] [MGI Ref ID J:107174]
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Okazaki T; Otaka Y; Wang J; Hiai H; Takai T; Ravetch JV; Honjo T. 2005. Hydronephrosis associated with antiurothelial and antinuclear autoantibodies in BALB/c-Fcgr2b-/-Pdcd1-/- mice. J Exp Med 202(12):1643-8. [PubMed: 16352741] [MGI Ref ID J:118829]
Park-Min KH; Serbina NV; Yang W; Ma X; Krystal G; Neel BG; Nutt SL; Hu X; Ivashkiv LB. 2007. FcgammaRIII-dependent inhibition of interferon-gamma responses mediates suppressive effects of intravenous immune globulin. Immunity 26(1):67-78. [PubMed: 17239631] [MGI Ref ID J:118322]
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Petkova SB; Akilesh S; Sproule TJ; Christianson GJ; Al Khabbaz H; Brown AC; Presta LG; Meng YG; Roopenian DC. 2006. Enhanced half-life of genetically engineered human IgG1 antibodies in a humanized FcRn mouse model: potential application in humorally mediated autoimmune disease. Int Immunol 18(12):1759-69. [PubMed: 17077181] [MGI Ref ID J:133048]
Petkova SB; Konstantinov KN; Sproule TJ; Lyons BL; Awwami MA; Roopenian DC. 2006. Human antibodies induce arthritis in mice deficient in the low-affinity inhibitory IgG receptor Fc gamma RIIB. J Exp Med 203(2):275-80. [PubMed: 16476768] [MGI Ref ID J:118959]
Pinheiro da Silva F; Aloulou M; Skurnik D; Benhamou M; Andremont A; Velasco IT; Chiamolera M; Verbeek JS; Launay P; Monteiro RC. 2007. CD16 promotes Escherichia coli sepsis through an FcR gamma inhibitory pathway that prevents phagocytosis and facilitates inflammation. Nat Med 13(11):1368-74. [PubMed: 17934470] [MGI Ref ID J:130075]
Pisitkun P; Deane JA; Difilippantonio MJ; Tarasenko T; Satterthwaite AB; Bolland S. 2006. Autoreactive B cell responses to RNA-related antigens due to TLR7 gene duplication. Science 312(5780):1669-72. [PubMed: 16709748] [MGI Ref ID J:109758]
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Currently there no information available for this strain. This may be due to the supply level of this strain.
| Pricing for USA, Canada and Mexico shipping destinations |
|
*Price(s) in US dollars ($)
Weeks of Age Price* Gender Cryorecovery Fee $1900.00
| Pricing for International shipping destinations |
|
*Price(s) in US dollars ($)
Weeks of Age Price* Gender Cryorecovery Fee $2470.00
| Standard Supply | Repository-Cryopreserved. Must Be Recovered. Please refer to pricing and supply notes for further information. |
|---|---|
| Supply Notes |
|
| The most appropriate controls for the Clcn1adr-mto5J mutant mice are littermates either wildtype or heterozygous for Clcn1adr-mto5J (and, ideally, matched for their Fcgr2btm1Rav genotype). Alternatively, B6129SF2 mice (Stock No. 101045) may be used as controls; the latter provide only an approximate genetic match to the mixed B6;129 background and lack the Fcgr2btm1Rav. | ||
| Considerations for Choosing Controls | ||
| USA, Canada and Mexico - Control Pricing Information for Genetically Engineered Mutant Strains. | ||
| International - Control Pricing Information for Genetically Engineered Mutant Strains. | ||
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