Former Names B6(C)-KitW-41J Gusbmps/SopJ (Changed: 25-JAN-07 ) Type Coisogenic; Mutant Strain; Spontaneous Mutation; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Species laboratory mouse H2 Haplotype b Generation F25p
Donating Investigator Brian Soper, The Jackson Laboratory
Mice homozygous for the "mps" (mucopolysaccharidosis type VII or MPS VII) mutation are devoid of expression of the lysosomal enzyme beta glucuronidase. Homozygous animals are viable, but females have a deficiency in lactation. Skeletal and connective tissue anomalies in both males and females are believed to prevent successful breeding. As this mutation is recessive, heterozygous mice are phenotypically similar to wildtype. Homozygotes exhibit short and thickened long bones (smaller than heterozygous or wildtype littermates), "pug type" appearance of the nose, hepatomegaly, splenomegaly, corneal clouding, and deafness. In appearance, homozygous KitW-41J mice are mostly white with black eyes and brown or grey spots. They are fertile, distinct from mice with other alleles of Kit, and have the impaired hemopoiesis causing mild, normochromic, macrocytic anemia. MPS VII mice are a model of the beta glucuronidase enzyme deficiency in humans called Sly Disease. They may be useful in developing new therapies (enzyme replacement, cell transplantation, gene therapy) broadly applicable to other lysosomal storage diseases. This strain, with combined KitW-41J and Gusbmps, provides a genetically myeloablated population in which to study stem cell engraftment and reconstitution.
This double mutant was generated by crossing B6.C-H2bm1/ByBir-Gusbmps/J (Stock No. 000256) and C57BL/6J-KitW-41J/J (Stock No. 000119). Progeny selected for the appropriate alleles were backcrossed to C57BL/6J-KitW-41J/J (Stock No. 000119) for 10 generations by Dr. Jane Barker at The Jackson Laboratory. The strain was donated to the Repository in 2007.
Strains carrying Gusbmps allele
006559 B6.C-H2-Kbm1/ByBir-Gusbmps/BrkJ 000256 B6.C-H2-Kbm1/ByBir-Gusbmps/J 006562 B6.CBy(Cg)-Gusbmps Gpi1a-m1J/BrkJ 002086 B6.Cg-Gusbmps Tg(Gussx)1Wat/J 006407 B6.Cg-Gusbmps/BrkJ 005053 NOD.Cg-Prkdcscid Gusbmps/SndsJ 001880 STOCK Gusbmps Tg(GUSB)4Sly/BirJView Strains carrying Gusbmps (7 strains)Strains carrying KitW-41J alleleView Strains carrying KitW-41J (2 strains)Strains carrying other alleles of Gusb
005643 B6.129X-Gusbtm1Sly/J 005644 B6.129X-Gusbtm3Sly/J 001603 B6.A-Gusba/J 001598 B6.C3-Gusbh/J 006557 B6.C3-Gusbmps-2J/BrkJ 001599 B6.CAST-Gusbcs/J 001604 B6.Cg-Ces1cb Ces1eh Gusbh/J 001605 B6.Cg-Gusbw12/CvJ 001608 B6.Cg-Gusbw26/CvJ 001602 B6.MOR-Gusbw5/CvJ 001597 B6.PAC-Gusbn/J 003525 C3H/HeOuJ-Gusbmps-2J/BrkJ 005322 C57BL/6J-Gusbmps-3J/JView Strains carrying other alleles of Gusb (13 strains)Strains carrying other alleles of Kit
016588 A.B6-KitW-v/BeiMmjax 000599 B6 x B6CBCa Aw-J/A-T(5;13)264Ca KitW-v/J 015813 B6.129S7-Kittm1Rosay/J 000495 B6.C-H38c/By-KitW-56J/J 000560 B6.C-H7b/By KitW-50J/J 000122 B6.C3-KitW-44J/J 000991 B6.C58-KitW-57J/J 002283 B6.Cg-KitW-19H/EiJ 000133 B6.Cg-KitW-24J/J 000139 B6.Cg-KitW-25J/J 012861 B6.Cg-KitW-sh/HNihrJaeBsmGlliJ 000164 B6.Cg-KitW/J 000194 B6.Cg-Lx KitW-v/J 000171 B6.D2-KitW-45J/J 001563 B6.D2-KitW-73J/J 001177 B6.LP-KitW-49J/J 025122 B6;D2-Tg(RP24-330G11-EGFP)1Mik/J 000350 B6By.Cg-KitW-v MitfMi-wh T/J 000627 C3H/HeJ-KitW-x/J 000847 C3Sn.B6-KitW-39J/J 000166 C57BL/6J-KitW-17J/J 000167 C57BL/6J-KitW-18J/J 000169 C57BL/6J-KitW-20J/J 000117 C57BL/6J-KitW-34J/J 000128 C57BL/6J-KitW-35J/J 000134 C57BL/6J-KitW-37J/J 000062 C57BL/6J-KitW-39J/J 000121 C57BL/6J-KitW-40J/J 000127 C57BL/6J-KitW-42J/J 000129 C57BL/6J-KitW-43J/J 000990 C57BL/6J-KitW-55J/J 001179 C57BL/6J-KitW-62J/J 021149 C57BL/6J-KitW-85J/GrsrJ 000049 C57BL/6J-KitW-v/J 000965 CBACa.C3-KitW-x/J 000092 FL/1Re-KitW/J 000993 NZB/BlNJ-KitW-59J/J 005051 STOCK KitW-sh/HNihrJaeBsmJ 000692 WB/ReJ KitW/J 100410 WBB6F1/J-KitW/KitW-v/JView Strains carrying other alleles of Kit (40 strains)
View Related Disease (OMIM) TermsRelated Disease (OMIM) Terms provided by MGIView Mammalian Phenotype TermsMammalian Phenotype Terms provided by MGIassigned by genotype
Gusbmps/Gusbmps KitW-41J/KitW-41JB6(C)-KitW-41J Gusbmps/BrkJ
- premature death
- average life span is approximately 26 weeks (MGI Ref ID J:58272)
- hematopoietic system phenotype
- decreased hematopoietic stem cell number
- severe deficiency of hematopoietic stem cells (MGI Ref ID J:58272)
- cellular phenotype
- abnormal lysosome morphology
- extensive lysosomal storage, evident histologically as large cleared foamy cells, in the liver, kidney, and spleen (MGI Ref ID J:58272)View Research ApplicationsResearch ApplicationsThis mouse can be used to support research in many areas including:
Immunology, Inflammation and Autoimmunity Research
Developmental Biology Research
Growth Defects (homozygous)
Behavioral and Learning Defects
Increased Tumor Incidence
Gonadal Tumors: ovarian
Color and White Spotting Defects
Developmental Biology Research
Neural Crest Defects
Endocrine Deficiency Research
Bone/Bone Marrow Defects
Immunology, Inflammation and Autoimmunity Research
Reproductive Biology Research
Developmental Defects Affecting Gonads
germ cell deficient
Immunology, Inflammation and Autoimmunity Research
Mast Cell Deficiency
|Allele Name||beta glucuronidase, mucopolysaccharidosis VII|
|Common Name(s)||Gus-b; MPS VII; asd; gusmps;|
|Mutation Made By||Brian Soper, The Jackson Laboratory|
|Strain of Origin||B6.C-H2-Kbm1/By|
|Gene Symbol and Name||Gusb, glucuronidase, beta|
|Gene Common Name(s)||AI747421; Ac2-223; BG; Gur; Gus; Gus-r; Gus-s; Gus-t; Gus-u; Gut; MPS7; adipose storage deficiency; asd; beta-glucuronidase regulator; beta-glucuronidase structural; beta-glucuronidase systemic regulator; beta-glucuronidase temporal; expressed sequence AI747421; g;|
|Molecular Note||A 1-bp deletion creates a frameshift mutation within exon 10, which introduces a premature stop codon at codon 497. [MGI Ref ID J:13207]|
|Allele Name||dominant spotting 41 Jackson|
|Common Name(s)||KitW41; W-41; W41;|
|Strain of Origin||C57BL/6J|
|Gene Symbol and Name||Kit, kit oncogene|
|Gene Common Name(s)||Bs; C-Kit; CD117; Dominant white spotting; Fdc; Gsfsco1; Gsfsco5; Gsfsow3; PBT; SCFR; SCO1; SCO5; SOW3; Ssm; Steel Factor Receptor; Tr-kit; W; belly-spot; dominant spotting; gsf spotted coat 1; gsf spotted coat 5; phenotype like Sl or W 3; spotted sterile male;|
|Molecular Note||Comparison of the coding sequence of this allele with normal c-kit indicated a G to A point mutation in the kinase domain at nucleotide 2519 that results in a valine to methionine substitution at amino acid 831. [MGI Ref ID J:10528]|
Soper BW; Duffy TM; Vogler CA; Barker JE. 1999. A genetically myeloablated MPS VII model detects the expansion and curative properties of as few as 100 enriched murine stem cells. Exp Hematol 27(11):1691-704. [PubMed: 10560917] [MGI Ref ID J:58272]
Gusbmps relatedKitW-41J related
Alfaro MP; Pagni M; Vincent A; Atkinson J; Hill MF; Cates J; Davidson JM; Rottman J; Lee E; Young PP. 2008. The Wnt modulator sFRP2 enhances mesenchymal stem cell engraftment, granulation tissue formation and myocardial repair. Proc Natl Acad Sci U S A 105(47):18366-71. [PubMed: 19017790] [MGI Ref ID J:142215]
Baldo G; Wu S; Howe RA; Ramamoothy M; Knutsen RH; Fang J; Mecham RP; Liu Y; Wu X; Atkinson JP; Ponder KP. 2011. Pathogenesis of aortic dilatation in mucopolysaccharidosis VII mice may involve complement activation. Mol Genet Metab 104(4):608-19. [PubMed: 21944884] [MGI Ref ID J:178881]
Barker JE; Deveau S; Lessard M; Hamblen N; Vogler C; Levy B. 2001. In Utero Fetal Liver Cell Transplantation without Toxic Irradiation Alleviates Lysosomal Storage in Mice with Mucopolysaccharidosis Type VII. Blood Cells Mol Dis 27(5):861-73. [PubMed: 11783949] [MGI Ref ID J:72147]
Bastedo L; Sands MS; Lambert DT; Pisa MA; Birkenmeier E; Chang PL. 1994. Behavioral consequences of bone marrow transplantation in the treatment of murine mucopolysaccharidosis type VII. J Clin Invest 94(3):1180-6. [PubMed: 8083358] [MGI Ref ID J:20455]
Beamer WG; Coleman DL. 1982. [Adipose storage deficiency (asd)]. Mouse News Lett 67:21. [MGI Ref ID J:13923]
Berry CL; Vogler C; Galvin NJ; Birkenmeier EH; Sly WS. 1994. Pathology of the ear in murine mucopolysaccharidosis type VII. Morphologic correlates of hearing loss. Lab Invest 71(3):438-45. [PubMed: 7933993] [MGI Ref ID J:20883]
Birkenmeier EH; Barker JE; Vogler CA; Kyle JW; Sly WS; Gwynn B; Levy B; Pegors C. 1991. Increased life span and correction of metabolic defects in murine mucopolysaccharidosis type VII after syngeneic bone marrow transplantation. Blood 78(11):3081-92. [PubMed: 1954394] [MGI Ref ID J:1655]
Birkenmeier EH; Davisson MT; Beamer WG; Ganschow RE; Vogler CA; Gwynn B; Lyford KA; Maltais LM; Wawrzyniak CJ. 1989. Murine mucopolysaccharidosis type VII. Characterization of a mouse with beta-glucuronidase deficiency. J Clin Invest 83(4):1258-6. [PubMed: 2495302] [MGI Ref ID J:9705]
Chen YH; Chang M; Davidson BL. 2009. Molecular signatures of disease brain endothelia provide new sites for CNS-directed enzyme therapy. Nat Med 15(10):1215-8. [PubMed: 19749771] [MGI Ref ID J:154308]
Daly TM; Vogler C; Levy B; Haskins ME; Sands MS. 1999. Neonatal gene transfer leads to widespread correction of pathology in a murine model of lysosomal storage disease. Proc Natl Acad Sci U S A 96(5):2296-300. [PubMed: 10051635] [MGI Ref ID J:53359]
Donsante A; Vogler C; Muzyczka N; Crawford JM; Barker J; Flotte T; Campbell-Thompson M; Daly T; Sands MS. 2001. Observed incidence of tumorigenesis in long-term rodent studies of rAAV vectors. Gene Ther 8(17):1343-6. [PubMed: 11571571] [MGI Ref ID J:71706]
Faust JR; Rodman JS; Daniel PF; Dice JF; Bronson RT. 1994. Two related proteolipids and dolichol-linked oligosaccharides accumulate in motor neuron degeneration mice (mnd/mnd), a model for neuronal ceroid lipofuscinosis. J Biol Chem 269(13):10150-5. [PubMed: 8144516] [MGI Ref ID J:17522]
Freeman BJ; Roberts MS; Vogler CA; Nicholes A; Hofling AA; Sands MS. 1999. Behavior and therapeutic efficacy of beta-glucuronidase-positive mononuclear phagocytes in a murine model of mucopolysaccharidosis type VII. Blood 94(6):2142-50. [PubMed: 10477745] [MGI Ref ID J:57636]
Frischmeyer-Guerrerio PA; Montgomery RA; Warren DS; Cooke SK; Lutz J; Sonnenday CJ; Guerrerio AL; Dietz HC. 2011. Perturbation of thymocyte development in nonsense-mediated decay (NMD)-deficient mice. Proc Natl Acad Sci U S A 108(26):10638-43. [PubMed: 21670277] [MGI Ref ID J:173551]
Ghodsi A; Stein C; Derksen T; Martins I; Anderson RD; Davidson BL. 1999. Systemic hyperosmolality improves beta-glucuronidase distribution and pathology in murine MPS VII brain following intraventricular gene transfer. Exp Neurol 160(1):109-16. [PubMed: 10630195] [MGI Ref ID J:58538]
Hofling AA; Vogler C; Creer MH; Sands MS. 2003. Engraftment of human CD34+ cells leads to widespread distribution of donor-derived cells and correction of tissue pathology in a novel murine xenotransplantation model of lysosomal storage disease. Blood 101(5):2054-63. [PubMed: 12406886] [MGI Ref ID J:109848]
Kyle JW; Birkenmeier EH; Gwynn B; Vogler C; Hoppe PC; Hoffmann JW; Sly WS. 1990. Correction of murine mucopolysaccharidosis VII by a human beta-glucuronidase transgene. Proc Natl Acad Sci U S A 87(10):3914-8. [PubMed: 2111021] [MGI Ref ID J:21256]
Li B; Sharpe EE; Maupin AB; Teleron AA; Pyle AL; Carmeliet P; Young PP. 2006. VEGF and PlGF promote adult vasculogenesis by enhancing EPC recruitment and vessel formation at the site of tumor neovascularization. FASEB J 20(9):1495-7. [PubMed: 16754748] [MGI Ref ID J:111344]
Meng XL; Shen JS; Kawagoe S; Ohashi T; Brady RO; Eto Y. 2010. Induced pluripotent stem cells derived from mouse models of lysosomal storage disorders. Proc Natl Acad Sci U S A 107(17):7886-91. [PubMed: 20385825] [MGI Ref ID J:159373]
Monroy MA; Ross FP; Teitelbaum SL; Sands MS. 2002. Abnormal osteoclast morphology and bone remodeling in a murine model of a lysosomal storage disease. Bone 30(2):352-9. [PubMed: 11856642] [MGI Ref ID J:109426]
Moullier P; Bohl D; Heard JM; Danos O. 1993. Correction of lysosomal storage in the liver and spleen of MPS VII mice by implantation of genetically modified skin fibroblasts [see comments] Nat Genet 4(2):154-9. [PubMed: 8348154] [MGI Ref ID J:11889]
Niermann GL; Watson GL. 1999. Growth hormone and insulin-like growth factor-I enhance beta-glucuronidase gene activation by androgen in mouse kidney. Mol Cell Endocrinol 153(1-2):47-55. [PubMed: 10459853] [MGI Ref ID J:56310]
O'Connor LH; Erway LC; Vogler CA; Sly WS; Nicholes A; Grubb J; Holmberg SW; Levy B; Sands MS. 1998. Enzyme replacement therapy for murine mucopolysaccharidosis type VII leads to improvements in behavior and auditory function. J Clin Invest 101(7):1394-400. [PubMed: 9525982] [MGI Ref ID J:46825]
Ohashi T; Watabe K; Uehara K; Sly WS; Vogler C; Eto Y. 1997. Adenovirus-mediated gene transfer and expression of human beta-glucuronidase gene in the liver, spleen, and central nervous system in mucopolysaccharidosis type VII mice. Proc Natl Acad Sci U S A 94(4):1287-92. [PubMed: 9037045] [MGI Ref ID J:38613]
Ohlemiller KK; Vogler CA; Roberts M; Galvin N; Sands MS. 2000. Retinal function is improved in a murine model of a lysosomal storage disease following bone marrow transplantation Exp Eye Res 71(5):469-81. [PubMed: 11040082] [MGI Ref ID J:66030]
Parente MK; Rozen R; Cearley CN; Wolfe JH. 2012. Dysregulation of gene expression in a lysosomal storage disease varies between brain regions implicating unexpected mechanisms of neuropathology. PLoS One 7(3):e32419. [PubMed: 22403656] [MGI Ref ID J:186852]
Poorthuis BJ; Romme AE; Willemsen R; Wagemaker G. 1994. Bone marrow transplantation has a significant effect on enzyme levels and storage of glycosaminoglycans in tissues and in isolated hepatocytes of mucopolysaccharidosis type VII mice. Pediatr Res 36(2):187-93. [PubMed: 7970933] [MGI Ref ID J:22149]
Sands MS; Birkenmeier EH. 1993. A single-base-pair deletion in the beta-glucuronidase gene accounts for the phenotype of murine mucopolysaccharidosis type VII. Proc Natl Acad Sci U S A 90(14):6567-71. [PubMed: 8101990] [MGI Ref ID J:13207]
Sands MS; Vogler C; Kyle JW; Grubb JH; Levy B; Galvin N; Sly WS; Birkenmeier EH. 1994. Enzyme replacement therapy for murine mucopolysaccharidosis type VII. J Clin Invest 93(6):2324-31. [PubMed: 8200966] [MGI Ref ID J:19122]
Sato T; Ikeda M; Yotsumoto S; Shimada Y; Higuchi T; Kobayashi H; Fukuda T; Ohashi T; Suda T; Ohteki T. 2013. Novel interferon-based pre-transplantation conditioning in the treatment of a congenital metabolic disorder. Blood 121(16):3267-73. [PubMed: 23412092] [MGI Ref ID J:196707]
Sferra TJ; Qu G; McNeely D; Rennard R; Clark KR; Lo WD; Johnson PR. 2000. Recombinant adeno-associated virus-mediated correction of lysosomal storage within the central nervous system of the adult mucopolysaccharidosis type VII mouse Hum Gene Ther 11(4):507-19. [PubMed: 10724030] [MGI Ref ID J:61251]
Simonaro CM; Ge Y; Eliyahu E; He X; Jepsen KJ; Schuchman EH. 2010. Involvement of the Toll-like receptor 4 pathway and use of TNF-alpha antagonists for treatment of the mucopolysaccharidoses. Proc Natl Acad Sci U S A 107(1):222-7. [PubMed: 20018674] [MGI Ref ID J:156466]
Skorupa AF; Fisher KJ; Wilson JM; Parente MK; Wolfe JH. 1999. Sustained production of beta-glucuronidase from localized sites after AAV vector gene transfer results in widespread distribution of enzyme and reversal of lysosomal storage lesions in a large volume of brain in mucopolysaccharidosis VII mice. Exp Neurol 160(1):17-27. [PubMed: 10630187] [MGI Ref ID J:58536]
Sly WS; Vogler C; Grubb JH; Zhou M; Jiang J; Zhou XY; Tomatsu S; Bi Y; Snella EM. 2001. Active site mutant transgene confers tolerance to human beta -glucuronidase without affecting the phenotype of MPS VII mice. Proc Natl Acad Sci U S A 98(5):2205-10. [PubMed: 11226217] [MGI Ref ID J:67876]
Stein CS; Ghodsi A; Derksen T; Davidson BL. 1999. Systemic and central nervous system correction of lysosomal storage in mucopolysaccharidosis type VII mice. J Virol 73(4):3424-9. [PubMed: 10074197] [MGI Ref ID J:53465]
Tomatsu S; Orii KO; Vogler C; Nakayama J; Levy B; Grubb JH; Gutierrez MA; Shim S; Yamaguchi S; Nishioka T; Montano AM; Noguchi A; Orii T; Kondo N; Sly WS. 2003. Mouse model of N-acetylgalactosamine-6-sulfate sulfatase deficiency (Galns-/-) produced by targeted disruption of the gene defective in Morquio A disease. Hum Mol Genet 12(24):3349-58. [PubMed: 14583446] [MGI Ref ID J:87155]
Vogler C; Sands M; Higgins A; Levy B; Grubb J; Birkenmeier EH; Sly WS. 1993. Enzyme replacement with recombinant beta-glucuronidase in the newborn mucopolysaccharidosis type VII mouse. Pediatr Res 34(6):837-40. [PubMed: 8108204] [MGI Ref ID J:22453]
Wolfe JH; Deshmane SL; Fraser NW. 1992. Herpesvirus vector gene transfer and expression of beta-glucuronidase in the central nervous system of MPS VII mice. Nat Genet 1(5):379-84. [PubMed: 1338772] [MGI Ref ID J:1832]
Woloszynek JC; Roberts M; Coleman T; Vogler C; Sly W; Semenkovich CF; Sands MS. 2004. Numerous transcriptional alterations in liver persist after short-term enzyme-replacement therapy in a murine model of mucopolysaccharidosis type VII. Biochem J 379(Pt 2):461-9. [PubMed: 14705966] [MGI Ref ID J:88886]
Benveniste P; Frelin C; Janmohamed S; Barbara M; Herrington R; Hyam D; Iscove NN. 2010. Intermediate-term hematopoietic stem cells with extended but time-limited reconstitution potential. Cell Stem Cell 6(1):48-58. [PubMed: 20074534] [MGI Ref ID J:157080]
Benz C; Copley MR; Kent DG; Wohrer S; Cortes A; Aghaeepour N; Ma E; Mader H; Rowe K; Day C; Treloar D; Brinkman RR; Eaves CJ. 2012. Hematopoietic stem cell subtypes expand differentially during development and display distinct lymphopoietic programs. Cell Stem Cell 10(3):273-83. [PubMed: 22385655] [MGI Ref ID J:185670]
Bowie MB; Kent DG; Copley MR; Eaves CJ. 2007. Steel factor responsiveness regulates the high self-renewal phenotype of fetal hematopoietic stem cells. Blood 109(11):5043-8. [PubMed: 17327414] [MGI Ref ID J:145423]
Bowie MB; McKnight KD; Kent DG; McCaffrey L; Hoodless PA; Eaves CJ. 2006. Hematopoietic stem cells proliferate until after birth and show a reversible phase-specific engraftment defect. J Clin Invest 116(10):2808-16. [PubMed: 17016561] [MGI Ref ID J:114978]
Cable J; Huszar D; Jaenisch R; Steel KP. 1994. Effects of mutations at the W locus (c-kit) on inner ear pigmentation and function in the mouse. Pigment Cell Res 7(1):17-32. [PubMed: 7521050] [MGI Ref ID J:21178]
Chen S; Burgin S; McDaniel A; Li X; Yuan J; Chen M; Khalaf W; Clapp DW; Yang FC. 2010. Nf1-/- Schwann cell-conditioned medium modulates mast cell degranulation by c-Kit-mediated hyperactivation of phosphatidylinositol 3-kinase. Am J Pathol 177(6):3125-32. [PubMed: 21037083] [MGI Ref ID J:167633]
D'Ascenzo M; Meacham C; Kitzman J; Middle C; Knight J; Winer R; Kukricar M; Richmond T; Albert TJ; Czechanski A; Donahue LR; Affourtit J; Jeddeloh JA; Reinholdt L. 2009. Mutation discovery in the mouse using genetically guided array capture and resequencing. Mamm Genome 20(7):424-36. [PubMed: 19629596] [MGI Ref ID J:151778]
Dykstra B; Kent D; Bowie M; McCaffrey L; Hamilton M; Lyons K; Lee SJ; Brinkman R; Eaves C. 2007. Long-term propagation of distinct hematopoietic differentiation programs in vivo. Cell Stem Cell 1(2):218-29. [PubMed: 18371352] [MGI Ref ID J:149712]
Dykstra B; Olthof S; Schreuder J; Ritsema M; de Haan G. 2011. Clonal analysis reveals multiple functional defects of aged murine hematopoietic stem cells. J Exp Med 208(13):2691-703. [PubMed: 22110168] [MGI Ref ID J:179054]
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Ingram DA; Yang FC; Travers JB; Wenning MJ; Hiatt K; New S; Hood A; Shannon K; Williams DA; Clapp DW. 2000. Genetic and biochemical evidence that haploinsufficiency of the Nf1 tumor suppressor gene modulates melanocyte and mast cell fates in vivo. J Exp Med 191(1):181-8. [PubMed: 10620616] [MGI Ref ID J:59248]
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Reith AD; Rottapel R; Giddens E; Brady C; Forrester L; Bernstein A. 1990. W mutant mice with mild or severe developmental defects contain distinct point mutations in the kinase domain of the c-kit receptor. Genes Dev 4(3):390-400. [PubMed: 1692559] [MGI Ref ID J:28221]
Sharma Y; Astle CM; Harrison DE. 2007. Heterozygous kit mutants with little or no apparent anemia exhibit large defects in overall hematopoietic stem cell function. Exp Hematol 35(2):214-220. [PubMed: 17258070] [MGI Ref ID J:123190]
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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 can be bred as heterozygotes. Homozygous Gusbmps females do not lactate. Homozygous Gusbmps males do not breed.
|Pricing for USA, Canada and Mexico shipping destinations|
Cryopreserved Mice - Ready for Recovery
Price (US dollars $) Cryorecovery* $2525.00
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* $3283.00
Cryorecovery - Standard.
Progeny testing is not required.
MICE, PRODUCTS AND SERVICES ARE PROVIDED “AS IS”. JACKSON EXTENDS NO WARRANTIES OF ANY KIND, EITHER EXPRESS, IMPLIED, OR STATUTORY, WITH RESPECT TO MICE, PRODUCTS OR SERVICES, INCLUDING ANY IMPLIED WARRANTY OF MERCHANTABILITY OR FITNESS FOR A PARTICULAR PURPOSE, OR ANY WARRANTY OF NON-INFRINGEMENT OF ANY PATENT, TRADEMARK, OR OTHER INTELLECTUAL PROPERTY RIGHTS.
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, JACKSON will, at its option, provide credit or replacement for the mice or product received or the services provided.
In no event shall JACKSON, 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 JACKSON, its agents or employees. Unless prohibited by law, in purchasing or receiving MICE, PRODUCTS or services from JACKSON, purchaser or recipient, or any party claiming by or through them, expressly releases and discharges JACKSON from all such causes of action or damages, and further agrees to defend and indemnify JACKSON from any costs or damages arising out of any third party claims.
MICE and PRODUCTS 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 JACKSON’s MICE, PRODUCTS or services. In addition, special terms and conditions of sale of certain MICE, PRODUCTS or services may be set forth separately in JACKSON 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 JACKSON, 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 JACKSON, 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 or services by JACKSON.