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Type Coisogenic; Mutant Strain; Spontaneous Mutation; Additional information on Genetically Engineered Mutant Mice. Species laboratory mouse Generation +N1 (03-JUL-05) Appearance
light agouti, affected
Related Genotype: A/A Lystbg-2J/Lystbg-2J
agouti, unaffected
Related Genotype: A/A Lystbg-2J/+Important Note
This strain is homozygous for the retinal degeneration allele Pde6brd1.Description
Mice homozygous for the beige 2J spontaneous mutation (Lystbg-2J) display characteristics very similar to the original beige mutation (Lystbg). The mouse phenotype closely resembles Chediak-Higashi syndrome 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 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 (Prkdcscid), in tissue graft and disease studies.
| Control | ||
|---|---|---|
| Heterozygote from the colony | ||
| 000659 C3H/HeJ | ||
| Considerations for Choosing Controls | ||
Strains carrying Lystbg-2J allele
000604 B6C3 a/A-T(10;13)199H +/+ Lystbg-J/J or Lystbg-2J/J View Strains carrying Lystbg-2J (1 strain)
Strains carrying Pde6brd1 allele
View Strains carrying Pde6brd1 (74 strains)
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 000629 C57BL/6J-Lystbg-J/J 000494 J.Cg-Oca2+ Tyr+ Lystbg/J 000269 SB/LeJ View Strains carrying other alleles of Lyst (5 strains)
Strains carrying other alleles of Pde6b
004297 B6.CXB1-Pde6brd10/J 002802 C3.BLiA Pde6b+-Krd/J 001979 C3A.BLiA-Pde6b+.O20-Prph2Rd2/J 001912 C3A.BLiA-Pde6b+/J 003648 C3Sn.BLiA-Pde6b+/Dn 004766 C57BL/6J-Pde6brd1-2J/J 004828 FVB.129P2-Pde6b+ Tyrc-ch/AntJ 004808 STOCK Mapttm1(EGFP)Klt Tg(MAPT)8cPdav/J View Strains carrying other alleles of Pde6b (8 strains)
JAX® NOTES, Spring 1991; 445. Why C57BL/6J-bgJ (beige) Mice are not Beige.
JAX® NOTES, Winter 1992; 448. The Beige (BgJ) Mutation.
View Research Applications
Research Applications
This mouse can be used to support research in many areas including:Lystbg-2J related
Pde6brd1 relatedCardiovascular 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
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)
Mouse/Human Gene Homologs
retinitis pigmentosa, autosomal recessive
Sensorineural Research
Retinal Degeneration
| Allele Symbol | Lystbg-2J | ||
|---|---|---|---|
| Allele Name | beige 2 Jackson | ||
| Allele Type | Spontaneous | ||
| Strain of Origin | Yellow stock | ||
| 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 | The molecular fault in the Lystbg-2J mutation may be an intronic retrotransposition event causing decreased transcription and mRNA instability (J:34205). | ||
| Molecular Note | Quantitative reverse transcription showed a five-fold decrease in Lyst mRNA in mice homozygous for this allele. The molecular basis for this decrease is not known. [MGI Ref ID J:34205] | ||
| Allele Symbol | Pde6brd1 | ||
| Allele Name | retinal degeneration 1 | ||
| Allele Type | Spontaneous | ||
| Common Name(s) | rd; rd-1; rd1; rodless retina; | ||
This strain will not have a genotyping protocol or one is not currently available.
Helpful Links
Optimizing PCR Protocols
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]
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]
Lystbg-2J relatedBarbosa MD; Nguyen QA; Tchernev VT; Ashley JA; Detter JC; Blaydes SM; Brandt SJ; Chotai D; Hodgman C; Solari RC; Lovett M; Kingsmore SF. 1996. Identification of the homologous beige and Chediak-Higashi syndrome genes. Nature 382(6588):262-5. [PubMed: 8717042] [MGI Ref ID J:34205]
Jadeski L; Hoffman-Goetz L. 1996. Exercise and in vivo natural cytotoxicity against tumour cells of varying metastatic capacity. Clin Exp Metastasis 14(2):138-44. [PubMed: 8605727] [MGI Ref ID J:33522]
Lane PW. 1962. bg-beige and bg<2J> - beige-2J Mouse News Lett 26:35. [MGI Ref ID J:65327]
Colony Maintenance
Diet Information LabDiet® 5K52/5K67
| 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 |
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| Important Note | This strain is homozygous for the retinal degeneration allele Pde6brd1. |
| Control | ||
|---|---|---|
| Heterozygote from the colony | ||
| 000659 C3H/HeJ | ||
| 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. | ||
Purchasing Information
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Contact Information
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Technical Support Email Form
| phone: | 207-288-6470 |
| fax: | 207-288-6655 |
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