Gene Symbol: Lyst
Description: lysosomal trafficking regulator
Alias: D13Sfk13, beige, lysosomal-trafficking regulator, CHS1 homolog
Species: mouse
Products:     Lyst

Top Publications

  1. Defosse D, Duray P, Johnson R. The NIH-3 immunodeficient mouse is a model for Lyme borreliosis myositis and carditis. Am J Pathol. 1992;141:3-10 pubmed
    ..deficiency due to the nude mutation, these mice have an x-linked defect affecting the B-cell maturation and the beige mutation resulting in the absence of NK cells. NIH-3 mice were susceptible to progressive infection with B...
  2. Lane P, Murphy E. Susceptibility to spontaneous pneumonitis in an inbred strain of beige and satin mice. Genetics. 1972;72:451-60 pubmed
    Among mice of strain SB/Le, homozygous for the mutant genes beige (bg), satin (sa), and white-bellied agouti (A(w)), 70% developed progressive pneumonitis by 6 months of age...
  3. Sweet H, Bronson R, Donahue L, Davisson M. Mesenchymal dysplasia: a recessive mutation on chromosome 13 of the mouse. J Hered. 1996;87:87-95 pubmed
    ..Mesenchymal dysplasia has been mapped to mouse chromosome (Chr) 13. These mapping crosses also confirmed that the Purkinje cell degeneration (pcd) mutation is on Chr 13. ..
  4. Barbosa M, Nguyen Q, Tchernev V, Ashley J, Detter J, Blaydes S, et al. Identification of the homologous beige and Chediak-Higashi syndrome genes. Nature. 1996;382:262-5 pubmed
    ..and from the lysosome and late endosome is defective in patients with Chediak-Higashi syndrome (CHS) and in mutant beige (bg) mice...
  5. Martin N, Jaubert J, Glaser P, Szatanik M, Guenet J. Genetic and physical delineation of the region overlapping the progressive motor neuropathy (pmn) locus on mouse chromosome 13. Genomics. 2001;75:9-16 pubmed
  6. Anderson M, Hawes N, Trantow C, Chang B, John S. Iris phenotypes and pigment dispersion caused by genes influencing pigmentation. Pigment Cell Melanoma Res. 2008;21:565-78 pubmed publisher
    ..Pigment dispersion was identified in several strains with mutant alleles known to influence melanosomes, including beige, light, and vitiligo...
  7. Perou C, Leslie J, Green W, Li L, Ward D, Kaplan J. The Beige/Chediak-Higashi syndrome gene encodes a widely expressed cytosolic protein. J Biol Chem. 1997;272:29790-4 pubmed
    The human autosomal recessive disorder Chediak-Higashi syndrome and its murine homologue beige are associated with the formation of giant lysosomes that cluster near the perinuclear region of cells...
  8. Trantow C, Mao M, Petersen G, Alward E, Alward W, Fingert J, et al. Lyst mutation in mice recapitulates iris defects of human exfoliation syndrome. Invest Ophthalmol Vis Sci. 2009;50:1205-14 pubmed publisher
    ..a distinctive pattern of iris transillumination defects that are recapitulated in Lyst mutant mice carrying the beige allele...
  9. Mosier D, Stell K, Gulizia R, Torbett B, Gilmore G. Homozygous scid/scid;beige/beige mice have low levels of spontaneous or neonatal T cell-induced B cell generation. J Exp Med. 1993;177:191-4 pubmed
    ..We have produced mice homozygous for both the scid and beige mutations. By contrast with C.B-17 scid mice, BALB/c scid.beige mice have a < 2% incidence of "leakiness...

More Information


  1. Shultz L, Ishikawa F, Greiner D. Humanized mice in translational biomedical research. Nat Rev Immunol. 2007;7:118-30 pubmed
  2. Perou C, Moore K, Nagle D, Misumi D, Woolf E, McGrail S, et al. Identification of the murine beige gene by YAC complementation and positional cloning. Nat Genet. 1996;13:303-8 pubmed
    The beige mutation is a murine autosomal recessive disorder, resulting in hypopigmentation, bleeding and immune cell dysfunction...
  3. Haliotis T, Ball J, Dexter D, Roder J. Spontaneous and induced primary oncogenesis in natural killer (NK)-cell-deficient beige mutant mice. Int J Cancer. 1985;35:505-13 pubmed
    ..These results suggest that a partial NK impairment in beige mutant mice early in life may lead to significantly greater rates of death with spontaneous malignant tumors late ..
  4. Mathiasen J, Raffa R, Vaught J. C57BL/6J-bgJ (beige) mice: differential sensitivity in the tail flick test to centrally administered mu- and delta-opioid receptor agonists. Life Sci. 1987;40:1989-94 pubmed
    ..and a selective delta-receptor agonist, [D-Pen2, L-Pen5]enkephalin (DPLPE), were determined in C57BL/6J-bgJ (beige) and control mice (CRS-CDl and C57BL/6By) using a standard tail-flick assay...
  5. Fang R, Ismail N, Walker D. Contribution of NK cells to the innate phase of host protection against an intracellular bacterium targeting systemic endothelium. Am J Pathol. 2012;181:185-95 pubmed publisher
    ..Furthermore, NK cells are involved in preventing rickettsial infection-induced endothelial cell damage, possibly via perforin production. ..
  6. Nonoyama S, Smith F, Bernstein I, Ochs H. Strain-dependent leakiness of mice with severe combined immune deficiency. J Immunol. 1993;150:3817-24 pubmed
    ..SCID) mice with that of two other strains of immune-deficient mice, the recently developed C3H SCID mice and the beige/nude/X-linked immune-deficient (BNX) mice. Detectable levels of serum lg (higher than 0...
  7. Kisucka J, Butterfield C, Duda D, Eichenberger S, Saffaripour S, Ware J, et al. Platelets and platelet adhesion support angiogenesis while preventing excessive hemorrhage. Proc Natl Acad Sci U S A. 2006;103:855-60 pubmed vivo approaches, the cornea micropocket assay and the Matrigel model, in four animal models: thrombocytopenic, Lyst(bg) (platelet storage pool deficiency), glycoprotein (GP) Ibalpha/IL4R transgenic (lacking extracellular GPIbalpha, ..
  8. Clark E, Shultz L, Pollack S. Mutations in mice that influence natural killer (NK) cell activity. Immunogenetics. 1981;12:601-13 pubmed
    ..Mutations at six loci that reduce NK-cell activity in the homozygous state were identified, including beige (bg), hairless (hr), motheaten (me), obese (ob), steel (Sl) and, to lesser extent, dominant spotting (W)...
  9. Jadeski L, Hoffman Goetz L. Exercise and in vivo natural cytotoxicity against tumour cells of varying metastatic capacity. Clin Exp Metastasis. 1996;14:138-44 pubmed
    ..fibroblasts (CIRAS 1 and CIRAS 3 cell lines) in mice with (C3H/He-bg2J/+) and without (C3H/HeJ) the beige (bg) mutation which produces impaired natural killer (NK) cell function...
  10. Petrovan R, Yuan Y, Curtiss L. Expression of the Lyst(beige) mutation is atheroprotective in chow-fed apolipoprotein E-deficient mice. J Lipid Res. 2008;49:429-37 pubmed
    Lyst(beige) mice crossed with hyperlipidemic low density lipoprotein receptor-deficient mice (BgLDLr(-/-)) display increased lesion area and a more stable lesion morphology...
  11. Chow L. Studies of virus-induced myocardial injury in mice: value of the scid mutation on different genetic backgrounds and combined with other mutations. Lab Anim Sci. 1993;43:133-5 pubmed
    ..The combined use of severe combined immunodeficient (scid) and doubly mutant scid/beige mice of different genetic backgrounds has provided a unique opportunity to study the consequences of viral ..
  12. Brandt E, Elliott R, Swank R. Defective lysosomal enzyme secretion in kidneys of Chediak-Higashi (beige) mice. J Cell Biol. 1975;67:774-88 pubmed
    The beige mouse is an animal model for the human Chediak-Higashi syndrome, a disease characterized by giant lysosomes in most cell types...
  13. Galli S, Hammel I. Unequivocal delayed hypersensitivity in mast cell-deficient and beige mice. Science. 1984;226:710-3 pubmed
    ..Because blood platelets represent another potential source of serotonin in delayed hypersensitivity responses, beige mice, whose platelets contain less than 1 percent of the normal levels of serotonin, were also examined...
  14. Marquis G, Montplaisir S, Pelletier M, Auger P, Lapp W. Genetics of resistance to infection with Candida albicans in mice. Br J Exp Pathol. 1988;69:651-60 pubmed
    To determine differences in susceptibility, 234 naive mice including xid and beige mutants were infected intravenously with Candida albicans and monitored with survival analysis and quantitative culture of the kidneys...
  15. Waggie K, Wu Owens J, Hollifield V, Hansen C. Lymphoblastic lymphoma in a colony of N:NIH(S)-bg-nu-xid mice. Lab Anim Sci. 1992;42:375-7 pubmed
    ..All of the above mice were homo- or hemizygous for the beige and X-linked immunodeficiency mutations. The average age of the mice was 8.3 months...
  16. Oliver C, Essner E. Distribution of anomalous lysosomes in the beige mouse: a homologue of Chediak-Higashi syndrome. J Histochem Cytochem. 1973;21:218-28 pubmed
  17. Novak E, McGarry M, Swank R. Correction of symptoms of platelet storage pool deficiency in animal models for Chediak-Higashi syndrome and Hermansky-Pudlak syndrome. Blood. 1985;66:1196-201 pubmed
    ..Reciprocal bone marrow transplants were carried out between normal C57BL/6J mice and two of these mutants, beige and pallid, in order to test whether the platelet defects are due to a defect in platelet progenitor cells or to ..
  18. Yoshizawa M, Okada T, Morikawa Y, Sasaki F, Kiso Y. Murine granulated metrial gland cell population in beige (bg/bg) and SCID (scid/scid) genotypes. J Vet Med Sci. 1994;56:415-6 pubmed
    ..Thus, the present results support the previous reports that GMG cells can differentiate normally in bg mice and that the GMG cell differentiation is not affected by functional T- nor B-cells. ..
  19. Gardi C, Cavarra E, Calzoni P, Marcolongo P, de Santi M, Martorana P, et al. Neutrophil lysosomal dysfunctions in mutant C57 Bl/6J mice: interstrain variations in content of lysosomal elastase, cathepsin G and their inhibitors. Biochem J. 1994;299 ( Pt 1):237-45 pubmed
    ..Another C57Bl/6J mutant with lysosomal abnormalities is the beige mouse...
  20. Zhang C, Cui Y, Houston S, Chang L. Protective immunity to HIV-1 in SCID/beige mice reconstituted with peripheral blood lymphocytes of exposed but uninfected individuals. Proc Natl Acad Sci U S A. 1996;93:14720-5 pubmed
    ..we have established a humanized SCID mouse infection model whereby T-, B-, and natural killer-cell defective SCID/beige mice that have been reconstituted with normal human PBLs can be infected with HIV-1...
  21. Ohsugi T, Kiuchi Y, Shimoda K, Oguri S, Maejima K. Translocation of bacteria from the gastrointestinal tract in immunodeficient mice. Lab Anim. 1996;30:46-50 pubmed
    ..the inhibition of translocation of bacteria from the gastrointestinal (GI) tract were investigated in SCID and beige mice after decontamination with oral antibiotics and colonization with Escherichia coli C25...
  22. Kveiborg M, Chiusaroli R, Sims N, Wu M, Sabatakos G, Horne W, et al. The increased bone mass in deltaFosB transgenic mice is independent of circulating leptin levels. Endocrinology. 2002;143:4304-9 pubmed
    ..This demonstration that restoring circulating levels of leptin in deltaFosB transgenic mice failed to rescue the bone phenotype further indicates that the marked increase in bone formation is autonomous to the osteoblast lineage. ..
  23. Barthold S, de Souza M. Exacerbation of Lyme arthritis in beige mice. J Infect Dis. 1995;172:778-84 pubmed
    ..was explored in disease-resistant C57/BL6 (B6) and -susceptible C3H/He (C3H) mice with and without the beige (bg) mutation, which is associated with granulocyte and NK cell dysfunction...
  24. Elin R, Edelin J, Wolff S. Infection and immunoglobulin concentrations in Chediak-Higashi mice. Infect Immun. 1974;10:88-91 pubmed
    ..CHS mice have an increased susceptibility to pyogenic infections, which is not due to immunoglobulin deficiency. These mice may provide a useful laboratory model for the study of increased susceptibility to infection. ..
  25. Kaido T, Gresser I, Maury C, Maunoury M, Vignaux F, Belardelli F. Sensitized T lymphocytes render DBA/2 beige mice responsive to IFN alpha/beta therapy of Friend erythroleukemia visceral metastases. Int J Cancer. 1993;54:475-81 pubmed
    ..v.) with FLC, but does not protect FLC-injected DBA/2 beige (bg/bg) mice...
  26. Kirkpatrick C, Farrell J. Leishmaniasis in beige mice. Infect Immun. 1982;38:1208-16 pubmed
    ..One group of mice was homozygous recessive for the beige gene (bg/bg)...
  27. Borges R, Jaen R, Freire F, Gomez J, Villafruela C, Yanes E. Morphological and functional characterization of beige mouse adrenomedullary secretory vesicles. Cell Tissue Res. 2001;304:159-64 pubmed
    We tested whether the giant secretory granules observed in the mast cells of the naturally occurring mutant beige mouse (BM) (C57BL/6N-bg) were also present in the adrenal chromaffin cells...
  28. Takeuchi K, Wood H, Swank R. Lysosomal elastase and cathepsin G in beige mice. Neutrophils of beige (Chediak-Higashi) mice selectively lack lysosomal elastase and cathepsin G. J Exp Med. 1986;163:665-77 pubmed
    ..of the two lysosomal serine proteinases, elastase, and cathepsin G, was found in neutrophils of four independent beige mutants...
  29. Chi E, Ignacio E, Lagunoff D. Mast cell granule formation in the beige mouse. J Histochem Cytochem. 1978;26:131-7 pubmed
    The formation of mast cell granules was studied in the beige mouse utilizing histochemistry and electron microscopy. The time and sequence of appearance of heparin, histamine and the chymotrypsin-like protease were normal...
  30. Lutzner M, Lowrie C, Jordan H. Giant granules in leukocytes of the beige mouse. J Hered. 1967;58:299-300 pubmed
  31. Gallo Hendrikx E, Percy D, Copps J, McKeown B, Quinton M, McMillan I, et al. Evaluation of three lines of immunodeficient mice for the study of spontaneous metastatic tumors. APMIS. 1999;107:245-56 pubmed
    ..We previously reported the establishment of transgenic mice that carried the SCID and/or beige mutations, eliminating B- and T-cell function and reducing lytic NK cell activity, respectively...
  32. Fernandez N, Lozier A, Flament C, Ricciardi Castagnoli P, Bellet D, Suter M, et al. Dendritic cells directly trigger NK cell functions: cross-talk relevant in innate anti-tumor immune responses in vivo. Nat Med. 1999;5:405-11 pubmed
    ..Thus, DC are involved in the interaction between innate and adaptive immune responses. ..
  33. Kuntz L, Jachez B, Roman D, Loor F. Modulation of adoptively transferred viable motheaten pathology in sublethally irradiated normal recipient mice by normal hematopoietic cells. Cell Immunol. 1993;146:215-21 pubmed
    ..This shows that the differentiation of mev HC in a bg or wild normal-type environment does not allow the maintenance of all mev HC-dependent abnormalities. ..
  34. Runkel F, Bussow H, Seburn K, Cox G, Ward D, Kaplan J, et al. Grey, a novel mutation in the murine Lyst gene, causes the beige phenotype by skipping of exon 25. Mamm Genome. 2006;17:203-10 pubmed
    ..mutant phenotype and the human Chediak-Higashi syndrome are caused by mutations in the murine Lyst (lysosomal trafficking regulator) gene and the human CHS gene, respectively...
  35. Winslow G, Yager E, Shilo K, Collins D, Chu F. Infection of the laboratory mouse with the intracellular pathogen Ehrlichia chaffeensis. Infect Immun. 1998;66:3892-9 pubmed
    ..B-17 and C57BL/6) mice cleared the bacteria within 10 days, but immunocompromised SCID and SCID/BEIGE mice developed persistent infection in the spleen, liver, peritoneal cavity, brain, lung, and bone marrow and ..
  36. Barbosa M, Barrat F, Tchernev V, Nguyen Q, Mishra V, Colman S, et al. Identification of mutations in two major mRNA isoforms of the Chediak-Higashi syndrome gene in human and mouse. Hum Mol Genet. 1997;6:1091-8 pubmed
    Chediak-Higashi syndrome is an autosomal recessive, immune deficiency disorder of human (CHS) and mouse (beige, bg) that is characterized by abnormal intracellular protein transport to, and from, the lysosome...
  37. Dove W, Clipson L, Gould K, Luongo C, Marshall D, Moser A, et al. Intestinal neoplasia in the ApcMin mouse: independence from the microbial and natural killer (beige locus) status. Cancer Res. 1997;57:812-4 pubmed
    ..In parallel, we have found that C57BL/6-ApcMin/+ mice mutated at the beige locus, which controls natural killer activity, are also unaltered in adenoma multiplicity.
  38. Pierce E, Carpenter K, Jakubzick C, Kunkel S, Flaherty K, Martinez F, et al. Therapeutic targeting of CC ligand 21 or CC chemokine receptor 7 abrogates pulmonary fibrosis induced by the adoptive transfer of human pulmonary fibroblasts to immunodeficient mice. Am J Pathol. 2007;170:1152-64 pubmed
    ..B-17 severe combined immunodeficiency (SCID)/beige (bg) mice...
  39. Percy D, Auger D, Croy B. Signs and lesions of experimental Sendai virus infection in two genetically distinct strains of SCID/beige mice. Vet Pathol. 1994;31:67-73 pubmed (SCID-beige) using C...
  40. Spritz R. Genetic defects in Chediak-Higashi syndrome and the beige mouse. J Clin Immunol. 1998;18:97-105 pubmed
    ..Disorders similar to human CHS occur in many mammalian species, the most important being the beige mouse, long considered a likely homologue of human CHS...
  41. Tischfield J, Ruddle F. Assignment of the gene for adenine phosphoribosyltransferase to human chromosome 16 by mouse-human somatic cell hybridization. Proc Natl Acad Sci U S A. 1974;71:45-9 pubmed
    ..This observation suggests that the human aprt gene can be assigned to chromosome 16. ..
  42. Clark R, Krishnan V, Schoof M, Rodriguez I, Theriault B, Chekmareva M, et al. Milky spots promote ovarian cancer metastatic colonization of peritoneal adipose in experimental models. Am J Pathol. 2013;183:576-91 pubmed publisher
    ..Our findings support a two-step model in which both milky spots and adipose have specific roles in colonization of the omentum by ovarian cancer cells. ..
  43. Roder J. The beige mutation in the mouse. I. A stem cell predetermined impairment in natural killer cell function. J Immunol. 1979;123:2168-73 pubmed
    A point mutation, called beige, on linkage group 14 in C57BL/6 mice leads to a marked impairment in natural killing and antibody-dependent, cell-mediated cytolysis (ADCC) of tumor cells...
  44. Kingsmore S, Barbosa M, Nguyen Q, Ashley J, Blaydes S, Tchernev V, et al. Physical mapping of the beige critical region on mouse chromosome 13. Mamm Genome. 1996;7:773-5 pubmed
  45. Lyon M, Meredith R. Muted, a new mutant affecting coat colour and otoliths of the mouse, and its position in linkage group XIV. Genet Res. 1969;14:163-6 pubmed
  46. Robison W, Kuwabara T. Light-induced alterations of retinal pigment epithelium in black, albino, and beige mice. Exp Eye Res. 1976;22:549-57 pubmed
  47. Song Y, Gan Y, Wang Q, Meng Z, Li G, Shen Y, et al. Enriching the Housing Environment for Mice Enhances Their NK Cell Antitumor Immunity via Sympathetic Nerve-Dependent Regulation of NKG2D and CCR5. Cancer Res. 2017;77:1611-1622 pubmed publisher
    ..T/B lymphocyte-deficient Rag1-/- mice, but was nearly eliminated in natural killer (NK) cell-deficient Beige mice or in antibody-mediated NK-cell-depleted mice, suggesting a predominant role of NK cells in enriched ..
  48. Takeuchi K, Toyohara H, Sakaguchi M. A hyperosmotic stress-induced mRNA of carp cell encodes Na(+)- and Cl(-)-dependent high affinity taurine transporter. Biochim Biophys Acta. 2000;1464:219-30 pubmed
    ..The Na(+)/Cl(-)hepatopancreas. Taurine transporter mRNA level increased up to 7.5-fold on raising the ambient osmolality from 300 to 450 mosmol/kgH(2)O. These data suggest the significant role of taurine as an osmolyte in carp cells. ..
  49. McGarry M, Reddington M, Novak E, Swank R. Survival and lung pathology of mouse models of Hermansky-Pudlak syndrome and Chediak-Higashi syndrome. Proc Soc Exp Biol Med. 1999;220:162-8 pubmed a pilot experiment involving several genetically defined singly and doubly mutant mouse HPS mutants and the beige CHS mutant to determine whether these parameters are altered in the mouse models...
  50. Takenaka K, Prasolava T, Wang J, Mortin Toth S, Khalouei S, Gan O, et al. Polymorphism in Sirpa modulates engraftment of human hematopoietic stem cells. Nat Immunol. 2007;8:1313-23 pubmed
    ..Thus, we have identified Sirpa polymorphism as a potent genetic determinant of the engraftment of human hematopoietic stem cells. ..
  51. Hearing V, Phillips P, Lutzner M. The fine structure of melanogenesis in coat color mutants of the mouse. J Ultrastruct Res. 1973;43:88-106 pubmed
  52. Swank R, Sweet H, Davisson M, Reddington M, Novak E. Sandy: a new mouse model for platelet storage pool deficiency. Genet Res. 1991;58:51-62 pubmed
    ..It represents the tenth example of a mouse mutant with simultaneous defects in melanosomes, lysosomes and/or platelet dense granules. ..
  53. Holcombe R, Stephenson D, Zweidler A, Stewart R, Chapman V, Seidman J. Linkage of loci associated with two pigment mutations on mouse chromosome 13. Genet Res. 1991;58:41-50 pubmed
    ..strains of mice were typed to map multiple markers in relation to two pigment mutations on mouse chromosome 13, beige (bg) and pearl (pe)...