Strain Name: |
B6.Cg-Lystbg/J |
|---|---|
Stock Number: |
000204 |
Availability: | Repository-Cryopreserved |
General Terms and Conditions |
| Genes & Alleles | Lyst; Lystbg; |
Product Information
Strain Details
Type JAX® GEMM® Strain - Congenic Additional information on JAX® GEMM® Strains. Type JAX® GEMM® Strain - Mutant Strain Species laboratory mouse Background Strain C57BL/6J Donor Strain Oak Ridge irr. Generation N14p Strain Description
Mice homozygous for the beige spontaneous mutation (Lystbg) are characterized by a condition that 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 (Prkdcscid), in tissue graft and disease studies.
Related Disease (OMIM) Terms |
Mammalian Phenotype Terms assigned by genotype |
Gene & Allele Details
| Allele Symbol | Lystbg | ||
|---|---|---|---|
| Allele Name | beige | ||
| Common Name(s) | 30B/22B; CHS mice; bg; | ||
| Strain of Origin | C3H/Rl | ||
| 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 original beige mutation was probably radiation-induced at Oak Ridge National Laboratory (J:29744). The Lystbg mutation contains an insertion causing abnormal splicing of mRNA products (J:33734).In Lystbg homozygotes, eye color is light at birth and varies from ruby to almost black in adults. Ear and tail pigmentation are reduced, and coat color is lighter (J:29744).The condition in homozygotes closely resembles Chediak-Higashi disease in man and similar conditions in mink and cattle (J:5078). In fact, mouse, mink, and human Chediak-Higashi fibroblasts fail to complement one another in cell fusion studies, indicating that the same gene is defective in all three species (J:16651). Pigment granules of both optic cup and neural crest derivatives, reduced in number, are much enlarged but have normal fine structure (J:5346). 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 (J:5538, J:5514); in type II pneumocytes (J:5992); in mast cells (J:5935); and in retinal pigment epithelium (J:5656). The giant lysosomes are formed by fusion of normal-sized ones (J:6629).Granulocytes of peritoneal exudate from Lystbg/Lystbg mice show defective chemotaxis and reduced bactericidal activity (J:5405). Beige mice are more susceptible than controls to pneumonitis (J:5311) and to various viral (J:6646), bacterial (J:5471), and parasitic (J:6946) infections. They have a severe deficiency of natural killer (NK) cells (J:6213), in common with several other pigment mutations that also affect lysosomal function (Hps1, Pldn, and Bloc1s3) (J:6801). Beige mice also havea defective cytotoxic T-cell and cytotoxic antibody response to allogeneic tumor cells (J:6213, J:6692). Syngeneic tumor cells grow better in beige mice than in littermate controls, an effect thought to be due to the deficiency of NK cells (J:6302, J:6301). The lysosomal serine proteases, elastase and cathepsin G, are profoundly decreased in peritoneal neutrophils of beige mice (J:8207), and the amount of plasminogen activator secreted by beige macrophages in culture is increased (J:7851). In the SB/Le-Lystbg strain, development of an autoimmune lupus-like syndrome in males is greatly retarded in beige homozygotes compared to rapid onset of autoimmunity in heterozygous +/Lystbg controls (J:30716). This may be a result of the immune defects cited above.The immunodeficiency of beige mutant mice has been used, especially in combination with the severe combined immunodeficiency (SCID) mutation (Prkdcscid), in tissue graft and disease studies. However, SCID-beige double mutant mice are highly susceptible to infection, even with organisms not usually regarded as mouse pathogens, and special care must be taken in maintaining these mice (J:4752).Beige mice have platelet storage pool deficiency (J:7327) similar to that observed in Hps1 and other pigment reduction mutations. The prolonged bleeding time and low dense granule content can be cured by transplantation of bone marrow from normal mice, and transferred to normal mice by transplantation of beige bone marrow (J:8056). Ithas been shown by use of concanavalin A cap formation that granulocytes of beige mice have defective membrane-related microtubular function, and that this as well as the granule abnormality can be corrected by promoters of cyclic GMP generation (J:5728).The abnormal lysosomes of kidney proximal tubules of beige mice show a striking accumulation of three lysosomal enzymes after androgen treatment, and the level of these enzymes in urine is lower than normal. Release of lysosomal contents into the tubulelumen appears to be defective (J:5590, J:6027). This also is paralleled by similar enzyme accumulation in other pigment mutants (see Hps1).Some of the pigment mutants that share the above lysosomal and platelet storage pool defects of the Lyst | ||
| Molecular Note | This allele is an insertion of a partial LINE 1 repetitive element into an intron of the gene. The insertion included only the most 3' 1097 bp of the element producing a frameshift mutation resulting in a truncated protein predicted to be missing the last 1442 amino acids. [MGI Ref ID J:33734] [MGI Ref ID J:41243] | ||
Related Strains
Strains carrying Lystbg allele
000494 J.Cg-Oca2+ Tyr+ Lystbg/J 000269 SB/LeJ View Strains carrying Lystbg (2 strains)
000604 B6C3 a/A-T(10;13)199H +/+ Lystbg-J/J or Lystbg-2J/J 000509 C3H/HeJ-Lystbg-2J/J 000629 C57BL/6J-Lystbg-J/J
Additional Web Information
Congenic Nomenclature
JAX® NOTES, Spring 1991; 445. Why C57BL/6J-bgJ (beige) Mice are not Beige.
JAX® NOTES, Winter 1992; 448. The Beige (BgJ) Mutation.
Research Applications
This mouse can be used to support research in many areas including:Lystbg 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
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)
References
Additional ReferencesPrice and Supply Information
| Strain Name: | B6.Cg-Lystbg/J |
| Stock Number: | 000204 |
Price Details
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Supply Details
| Standard Supply | Repository-Cryopreserved. Must Be Recovered. Please refer to pricing and supply notes for further information. |
|---|---|
| Supply Notes |
Cryorecovery - Standard. The recovery process begins when a signed agreement form is returned to the Customer Service Department after order placement. Although results vary by strain, at least two males and two females (two pairs) will be provided, typically within 15 weeks of our receipt of the signed agreement form. If the first recovery attempt is unsuccessful or only one pair is recovered, a second recovery will be done, extending the delivery time to approximately 25 weeks. At least one member of each pair will be of known genotype and will carry the mutation if it is a mutant strain. Please note that pairs may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation of the strain. Mating schemes are sometimes modified for successful cryopreservation. Price represents a repository maintenance fee, which includes the cost of recovery of the strain from the cryopreservation resource and the periodic replacement of the frozen embryos used for recovery. Cryorecovery to establish a Dedicated Supply for greater quantities of mice. |
| Licensing | See General Terms and Conditions below |
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