Description

Strain Information

Type Coisogenic; Mutant Strain; Spontaneous Mutation; Additional information on Genetically Engineered Mutant Mice. Mating System Homozygote x Homozygote         (Female x Male) Species laboratory mouse Generation N11F6 (03-JAN-08)

Appearance
dark gray, affected
Related Genotype: a/a Lyst^bg-J/Lyst^bg-J

black, unaffected
Related Genotype: a/a Lyst^bg-J/+

Description
Mice homozygous for the beige-J spontaneous mutation (Lyst^bg-J) are identical to the original beige mutation (Lyst^bg). The phenotype closely resembles Chediak-Higashi disease in man and similar conditions in mink and cattle. Abnormal giant lysosomal granules occur in all tissues with granule-containing cells, including granulocytes, lymphocytes, cells of the liver, kidney, central nervous system, pancreas, and thyroid, and the ducts of most glands; in type II pneumocytes; in mast cells; and in retinal pigment epithelium. Granulocytes from beige homozygous mutant mice mice show defective chemotaxis and reduced bactericidal activity. Beige mice are more susceptible than controls to pneumonitis and to various viral, bacterial, and parasitic infections. Natural killer (NK) cells from beige mice exhibit decreased endogenous cytotoxic activity. Beige mice also have a defective cytotoxic T-cell and cytotoxic antibody response to allogeneic tumor cells. Syngeneic tumor cells grow better in beige mice than in littermate controls, an effect thought to be due to the deficiency of NK cells. Beige mice have platelet storage pool deficiency, leading to a prolonged bleeding time. The immunodeficiency of beige mutant mice has been used, especially in combination with the scid mutation (Prkdc^scid), in tissue graft and disease studies.

Control Information

	Control
	000664 C57BL/6J
Considerations for Choosing Controls

Related Strains

Strains carrying   Lyst^bg-J allele

000604

B6C3 a/A-T(10;13)199H +/+ Lystbg-J/J or Lystbg-2J/J

View Strains carrying   Lyst^bg-J     (1 strain)

Strains carrying other alleles of Lyst

000204	B6.Cg-Lystbg/J
000604	B6C3 a/A-T(10;13)199H +/+ Lystbg-J/J or Lystbg-2J/J
000509	C3H/HeJ-Lystbg-2J/J
000494	J.Cg-Oca2+ Tyr+ Lystbg/J
000269	SB/LeJ

View Strains carrying other alleles of Lyst     (5 strains)

Additional Web Information

Genetic Quality Control Annual Report
JAX^® NOTES, Spring 1991; 445. Why C57BL/6J-bg^J (beige) Mice are not Beige.
JAX^® NOTES, Spring 2003; 489. Role of NK and NKT Cells in Immunity and Disease.
JAX^® NOTES, Winter 1992; 448. The Beige (Bg^J) Mutation.

Phenotype

Phenotype Information

View Mammalian Phenotype Terms

Mammalian Phenotype Terms

      assigned by genotype

Lyst^bg-J/Lyst^bg-J

        C57BL/6J-Lyst^bg-J/J

• immune system phenotype
• abnormal granulocyte morphology (MGI Ref ID J:34814)
  • granulocytes are characterized by giant lysosomal sudanophilic granules
• abnormal neutrophil morphology (MGI Ref ID J:34814)
  • neutrophil lysosomal extracts have decreased elastase activity
• decreased macrophage cell number (MGI Ref ID J:34814)
  • F4/80+ splenic macrophages are decreased in comparison to control (3.8% vs. 8.8%)
• impaired NK cell cytolysis (MGI Ref ID J:34814)
  • NK cell activity is decreased in comparison to B6 control
• hematopoietic system phenotype
• abnormal granulocyte morphology (MGI Ref ID J:34814)
  • granulocytes are characterized by giant lysosomal sudanophilic granules
• abnormal neutrophil morphology (MGI Ref ID J:34814)
  • neutrophil lysosomal extracts have decreased elastase activity
• abnormal platelet physiology (MGI Ref ID J:7327)
  • platelet ATP and ADP levels are much lower than in C57BL/6J controls
• decreased platelet serotonin level (MGI Ref ID J:7327)
  • less than 1% of normal platelet serotonin levels
• decreased macrophage cell number (MGI Ref ID J:34814)
  • F4/80+ splenic macrophages are decreased in comparison to control (3.8% vs. 8.8%)
• platelet storage pool deficiency (MGI Ref ID J:7327)
• homeostasis/metabolism phenotype
• abnormal platelet physiology (MGI Ref ID J:7327)
  • platelet ATP and ADP levels are much lower than in C57BL/6J controls
• decreased platelet serotonin level (MGI Ref ID J:7327)
  • less than 1% of normal platelet serotonin levels
• increased bleeding time (MGI Ref ID J:7327)
  • bleed time averaging over 11 minutes after tail nick is much greater than the 3.8 minutes for C57BL/6J controls
• platelet storage pool deficiency (MGI Ref ID J:7327)
• cellular phenotype
• decreased lysosomal enzyme secretion (MGI Ref ID J:7327)
  • thrombin stimulation of platelets results in approximately half normal levels of secretion of beta-glucuronidase and beta-galactosidase

View Research Applications

Research Applications

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

Lyst^bg-J related

Cardiovascular Research
Atherosclerosis

Dermatology Research
Color and White Spotting Defects

Hematological Research
Immunological Defects
Platelet Defects (platelet storage pool deficiency)

Immunology and Inflammation Research
CD Antigens, Antigen Receptors, and Histocompatibility Markers
Immunodeficiency (NK Cell and Cd8 T Cell defects)
Immunodeficiency Associated with Other Defects
Intracellular Signaling Molecules

Internal/Organ Research
Kidney Defects (lysosomal enzyme abnormalities)

Metabolism Research

Mouse/Human Gene Homologs
Chediak-Higashi syndrome

Research Tools
Immunology and Inflammation Research (NK Cell Deficiency)

Genes & Alleles

Gene & Allele Information

Allele Symbol		Lystbg-J
Allele Name		beige Jackson
Allele Type		Spontaneous
Common Name(s)		bgj;
Strain of Origin		C57BL/6J
Gene Symbol and Name		Lyst, lysosomal trafficking regulator
Chromosome		13
Gene Common Name(s)		Beige; CHS; CHS1; D13Sfk13; DNA segment, Chr 13, Stephen F. Kingsmore 13; beige; bg;
General Note		This remutation occurred spontaneously in the C57BL/6J strain at The Jackson Laboratory (J:5311). It has essentially identical effects to the original Lystbg mutation, and has been used extensively in defining locus effects.
Molecular Note		This allele is defined by a noncomplementation test with Lystbg. Although mice homozygous for this allele show a decrease in Lyst mRNA in the liver compared to normal, the molecular basis for this allele is not yet known. [MGI Ref ID J:7539]

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)

Gallin JI; Bujak JS; Patten E; Wolff SM. 1974. Granulocyte function in the Chediak-Higashi syndrome of mice. Blood 43(2):201-6. [PubMed: 4589319]  [MGI Ref ID J:5405]

Perou CM; Moore KJ; Nagle DL; Misumi DJ; Woolf EA; McGrail SH; Holmgren L; Brody TH; Dussault BJ Jr; Monroe CA; Duyk GM; Pryor RJ; Li L; Justice MJ; Kaplan J. 1996. Identification of the murine beige gene by YAC complementation and positional cloning. Nat Genet 13(3):303-8. [PubMed: 8673129]  [MGI Ref ID J:33734]

Roder JC. 1979. The beige mutation in the mouse. I. A stem cell predetermined impairment in natural killer cell function. J Immunol 123(5):2168-73. [PubMed: 489978]  [MGI Ref ID J:6213]

Additional References

Chiang EY; Stroynowski I. 2004. A nonclassical MHC class I molecule restricts CTL-mediated rejection of a syngeneic melanoma tumor. J Immunol 173(7):4394-401. [PubMed: 15383569]  [MGI Ref ID J:93729]

Glass WG; Subbarao K; Murphy B; Murphy PM. 2004. Mechanisms of host defense following severe acute respiratory syndrome-coronavirus (SARS-CoV) pulmonary infection of mice. J Immunol 173(6):4030-9. [PubMed: 15356152]  [MGI Ref ID J:92752]

Schiller NK; Black AS; Bradshaw GP; Bonnet DJ; Curtiss LK. 2004. Participation of macrophages in atherosclerotic lesion morphology in LDLr-/- mice. J Lipid Res 45(8):1398-409. [PubMed: 15175354]  [MGI Ref ID J:91367]

Wu HS; Kolonoski P; Chang YY; Bermudez LE. 2000. Invasion of the brain and chronic central nervous system infection after systemic Mycobacterium avium complex infection in mice. Infect Immun 68(5):2979-84. [PubMed: 10768998]  [MGI Ref ID J:61694]

Lyst^bg-J related

Bumgardner GL; Gao D; Li J; Baskin JH; Heininger M; Orosz CG. 2000. Rejection responses to allogeneic hepatocytes by reconstituted SCID mice, CD4, KO, and CD8 KO mice. Transplantation 70(12):1771-80. [PubMed: 11152110]  [MGI Ref ID J:66571]

Bussiere JL; Adler MW; Rogers TJ; Eisenstein TK. 1993. Effects of in vivo morphine treatment on antibody responses in C57BL/6 bgJ/bgJ (beige) mice. Life Sci 52(4):PL43-8. [PubMed: 8421432]  [MGI Ref ID J:4023]

Carlson GA; Marshall ST; Truesdale AT. 1984. Adaptive immune defects and delayed rejection of allogeneic tumor cells in beige mice. Cell Immunol 87(2):348-56. [PubMed: 6467382]  [MGI Ref ID J:7539]

Chiang EY; Stroynowski I. 2004. A nonclassical MHC class I molecule restricts CTL-mediated rejection of a syngeneic melanoma tumor. J Immunol 173(7):4394-401. [PubMed: 15383569]  [MGI Ref ID J:93729]

Christianson SW; Greiner DL; Schweitzer IB; Gott B; Beamer GL; Schweitzer PA; Hesselton RM; Shultz LD. 1996. Role of natural killer cells on engraftment of human lymphoid cells and on metastasis of human T-lymphoblastoid leukemia cells in C57BL/6J-scid mice and in C57BL/6J-scid bg mice. Cell Immunol 171(2):186-99. [PubMed: 8806787]  [MGI Ref ID J:34814]

Davis SG; Lyerla TA. 1997. The effect of lysosomotropic amines on beige mouse cells. Exp Cell Res 237(1):242-5. [PubMed: 9417888]  [MGI Ref ID J:44747]

Dove WF; Clipson L; Gould KA; Luongo C; Marshall DJ; Moser AR; Newton MA; Jacoby RF. 1997. Intestinal neoplasia in the ApcMin mouse: independence from the microbial and natural killer (beige locus) status. Cancer Res 57(5):812-4. [PubMed: 9041176]  [MGI Ref ID J:38777]

Gardi C; Cavarra E; Calzoni P; Marcolongo P; de Santi M; Martorana PA; Lungarella G. 1994. Neutrophil lysosomal dysfunctions in mutant C57 Bl/6J mice: interstrain variations in content of lysosomal elastase, cathepsin G and their inhibitors. Biochem J 299(Pt 1):237-45. [PubMed: 8166647]  [MGI Ref ID J:17583]

Glass WG; Subbarao K; Murphy B; Murphy PM. 2004. Mechanisms of host defense following severe acute respiratory syndrome-coronavirus (SARS-CoV) pulmonary infection of mice. J Immunol 173(6):4030-9. [PubMed: 15356152]  [MGI Ref ID J:92752]

Gross SK; Lyerla TA; Williams MA; McCluer RH. 1992. The accumulation and metabolism of glycosphingolipids in primary kidney cell cultures from beige mice. Mol Cell Biochem 118(1):61-6. [PubMed: 1488056]  [MGI Ref ID J:21419]

Kveiborg M; Chiusaroli R; Sims NA; Wu M; Sabatakos G; Horne WC; Baron R. 2002. The increased bone mass in deltaFosB transgenic mice is independent of circulating leptin levels. Endocrinology 143(11):4304-9. [PubMed: 12399426]  [MGI Ref ID J:80469]

Lane PW. 1962. bg-beige and bg<2J> - beige-2J Mouse News Lett 26:35.  [MGI Ref ID J:65327]

Lane PW; Murphy ED. 1972. Susceptibility to spontaneous pneumonitis in an inbred strain of beige and satin mice. Genetics 72(3):451-60. [PubMed: 4643821]  [MGI Ref ID J:5311]

Marquis G; Montplaisir S; Pelletier M; Auger P; Lapp WS. 1988. Genetics of resistance to infection with Candida albicans in mice. Br J Exp Pathol 69(5):651-60. [PubMed: 3058198]  [MGI Ref ID J:27324]

Novak EK; Hui SW; Swank RT. 1984. Platelet storage pool deficiency in mouse pigment mutations associated with seven distinct genetic loci. Blood 63(3):536-44. [PubMed: 6696991]  [MGI Ref ID J:7327]

Paigen B; Holmes PA; Novak EK; Swank RT. 1990. Analysis of atherosclerosis susceptibility in mice with genetic defects in platelet function. Arteriosclerosis 10(4):648-52. [PubMed: 2369371]  [MGI Ref ID J:29748]

Pak MW; Giolli RA; Pinto LH; Mangini NJ; Gregory KM; Vanable JW Jr. 1987. Retinopretectal and accessory optic projections of normal mice and the OKN-defective mutant mice beige, beige-J, and pearl. J Comp Neurol 258(3):435-46. [PubMed: 3584547]  [MGI Ref ID J:29747]

Perou CM; Leslie JD; Green W; Li L; Ward DM; Kaplan J. 1997. The Beige/Chediak-Higashi syndrome gene encodes a widely expressed cytosolic protein. J Biol Chem 272(47):29790-4. [PubMed: 9368050]  [MGI Ref ID J:44170]

Petrovan RJ; Yuan Y; Curtiss LK. 2008. Expression of the Lystbeige mutation is atheroprotective in chow-fed apolipoprotein E-deficient mice. J Lipid Res 49(2):429-37. [PubMed: 17982137]  [MGI Ref ID J:131876]

Prigent D; Trancart MM; Seed MP; Willoughby DA. 1996. Proteoglycan degrading activity in granulomatous inflammation: comparison between the C57b1/6 and C57bg/bg mouse. Inflamm Res 45(10):494-8. [PubMed: 8912013]  [MGI Ref ID J:36682]

Schiller NK; Black AS; Bradshaw GP; Bonnet DJ; Curtiss LK. 2004. Participation of macrophages in atherosclerotic lesion morphology in LDLr-/- mice. J Lipid Res 45(8):1398-409. [PubMed: 15175354]  [MGI Ref ID J:91367]

Schiller NK; Boisvert WA; Curtiss LK. 2002. Inflammation in atherosclerosis: lesion formation in LDL receptor-deficient mice with perforin and Lyst(beige) mutations. Arterioscler Thromb Vasc Biol 22(8):1341-6. [PubMed: 12171798]  [MGI Ref ID J:103214]

Silvers WK. 1979. The Coat Colors of Mice; A Model for Mammalian Gene Action and Interaction. In: The Coat Colors of Mice. Springer-Verlag, New York.  [MGI Ref ID J:78801]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           AX30

Colony Maintenance

Mating System	Homozygote x Homozygote         (Female x Male)
Diet Information	LabDiet® 5K52/5K67

Purchasing information

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

Pricing

Supply Details

Standard Supply	Level 4. Up to 10 mice. Larger quantities or custom orders arranged upon request. Expected delivery up to one to three months.
Supply Notes	Shipped at a specific age in weeks. Mice at a precise age in days, littermates and retired breeders are also available. Strains that must be genotyped are not available until five to seven weeks of age. Genomic DNA is available for this strain from the Mouse DNA Resource.

Control Information

	Control
	000664 C57BL/6J
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.

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.

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

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