Strain Name: |
SB/LeJ |
|---|---|
Stock Number: |
000269 |
Availability: | Repository-Cryopreserved |
General Terms and Conditions |
| Former Name |
SB/LeJ-Lystbg (Changed: 27-MAR-08
) |
| Strain Common Names | satin beige; |
| Genes & Alleles | Foxq1; Foxq1sa; Lyst; Lystbg; Pde6b; Pde6brd1; |
Product Information
Strain Details
Type Inbred Strain Additional information on Inbred Strains. Type JAX® GEMM® Strain - Mutant Strain Additional information on JAX® GEMM® Strains. Type JAX® GEMM® Strain - Radiation Induced Mutation Type JAX® GEMM® Strain - Spontaneous Mutation Species laboratory mouse H2 Haplotype b Generation F83p Appearance
pale grey
Related Genotype: Aw/Aw Lystbg Foxq1sa/Lystbg Foxq1saImportant Note
This strain is homozygous for Lystbg, Foxq1sa and the retinal degeneration allele Pde6brd1. See article "Genetic Background Effects: Can Your Mice See?", JAX Notes Spring 2002, No. 485.Strain Description
The SB/Le inbred strain is homozygous for both the satin (Foxq1sa) and beige (Lystbg) mutations. Most of the immunological phenotype is due to the beige mutation. Homozygous mutant beige mice 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 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. They have a severe deficiency of natural killer (NK) cells. 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. SB/Le mice are also homozygous for white-bellied agouti (Aw) and the retinal degeneration 1 mutation (Pde6brd1). The retinal degeneration 1 mutation leads to early blindness (by weaning). Quantitative RT-PCR comparing Lyst mRNA from mice with the Lystbg, Lystbg-J, or Lystbg-2J alleles has shown that mice with the Lystbg-2J allele have the greatest reduction in mRNA, and mice with the Lystbg-J allele have the least reduction (Barbosa et al. Nature 1996 382:262-265). The precise function of the Lyst protein remains undetermined. Homology searches and defective Concanabalin A induced capping have lead to the postulate that Lyst is involved in microtubule function.Strain Development
Both the satin (Foxq1sa) and the original beige (Lystbg) recessive mutations arose at Oak Ridge National Laboratory in backcross test matings from T stock female x irradiated (101/Rl x C3H/Rl)F1 male. T stock was a multiple-recessive tester strain that contained several backgrounds including C57BL10/Rl. The two separate mutations, Foxq1sa and bg, were bred together and homozygous Aw/Aw Foxq1sa bg/Foxq1sa bg mice on a mixed background were received at The Jackson Laboratory in 1961 and sibling mated thereafter. In 1963 this breeding was at F7, in 1967 it reached F20, and in 1971 the strain was re-named SB/LeJ, an inbred strain (Lane et al., 1972 Genetics 71:451-460). The Lystbg allele was found to have arisen from the retrotransposition of a LINE1 element resulting in a cDNA sequence which predicts a truncated Lyst protein product (Perou et al., Genomics 1997; 42:366-368). This insertion occurred in the C3H derived chromosome rather than the 101/R1 derived chromosome in the irradiated founder (101/Rl x C3H/Rl)F1 male (Perou et al., Nature Genetics 1996; 13:303-308). Subsequent sponta neous mutations of Lyst have been identified. It is worthwhile to note that Stock No. 000204, the C57BL/6J congenic of Lystbg named B6.Cg-Lystbg/J, and Stock No. 000629, the spontaneous mutation on C57BL/6J named C57BL/6J-Lystbg-J/J are both offered by The Jackson Laboratory and should not be confused.
Gene & Allele Details
| Allele Symbol | Foxq1sa | ||
|---|---|---|---|
| Allele Name | satin | ||
| Common Name(s) | sa; | ||
| Strain of Origin | (101/Rl x C3H/Rl)F1 | ||
| Gene Symbol and Name | Foxq1, forkhead box Q1 | ||
| Chromosome | 13 | ||
| Gene Common Name(s) | HFH-1; HFH1; HNF-3/forkhead homolog 1; HNF-3/forkhead homolog 1 like; Hfh1; Hfh1l; sa; satin; | ||
| Molecular Note | This allele occured in descendants of a gamma irradiation experiment. The underlying mutation was identified as a 67 bp intragenic deletion. The deletion causes a frame shift resulting in a truncated 376 amino acid protein but does not affect the first 228 residues, including the characteristic WH DNA-binding domain. The mutant protein is predicted to bind to its DNA targets but fails at transcriptional activation or repression because the specific C-terminal domains are missing. [MGI Ref ID J:75422] | ||
| 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] | ||
| Allele Symbol | Pde6brd1 | ||
| Allele Name | retinal degeneration 1 | ||
| Common Name(s) | rd; rd-1; rd1; rodless retina; | ||
| Gene Symbol and Name | Pde6b, phosphodiesterase 6B, cGMP, rod receptor, beta polypeptide | ||
| Chromosome | 5 | ||
| Gene Common Name(s) | CSNB3; PDEB; Pdeb; RP40; nmf137; phosphodiesterase, cGMP, rod receptor, beta polypeptide; r; rd; rd-1; rd1; rd10; retinal degeneration; retinal degeneration 1; retinal degeneration 10; | ||
| Molecular Note | Two mutations have been identified in rd1 mice. A murine leukimia virus (Xmv-28) insertion in reverse orientation in intron 1 is found in all mouse strains with the rd1 phenotype. Further, a nonsense mutation (C to A transversion) in codon 347 that results in a truncation eliminating more than half of the predicted encoded protein, including the catalytic domain has also been identified in all rd1 strains of mice. A specific degradation of mutant transcript during or after pre-mRNA splicing is suggested. [MGI Ref ID J:11513] [MGI Ref ID J:4366] [MGI Ref ID J:51361] | ||
Control Information
| Control | ||
|---|---|---|
| None Available | ||
| Considerations for Choosing Controls | ||
Related Strains
Strains carrying Lystbg allele
000204 B6.Cg-Lystbg/J 000494 J.Cg-Oca2+ Tyr+ Lystbg/J View Strains carrying Lystbg (2 strains)
003896 MRL/MpJ Faslpr-Foxq1sa-J/J
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
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
Phenotypic Data
Mouse Phenome Database
Festing Inbred Strain Characteristics: SB
Additional Web Information
JAX Notes, Spring 2002; 485. Genetic Background Effects: Can Your Mice See?
Research Applications
This mouse can be used to support research in many areas including:Foxq1sa related
Lystbg relatedDermatology Research
Skin and Hair Texture Defects
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
References
Additional ReferencesPrice and Supply Information
| Strain Name: | SB/LeJ |
| Stock Number: | 000269 |
Price Details
IMPORTANT NOTE: Prices are based on shipping destination. To view prices, select your shipping destination.
*NO Shipping Destination selected!
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 |
| Control Information | View Control Information in Strain Details. |
General Terms and Conditions
View JAX® Mice & Services Conditions 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.Ordering and Purchasing Information
Purchasing Information
JAX® Mice Orders
Surgical Services
Contact Information
Orders & Technical Support
Tel: 800.422.6423 or 207.288.5845
Fax: 207.288.6150
Technical Support Email Form