Gene Symbol: Gusb
Description: glucuronidase, beta
Alias: AI747421, Gur, Gus, Gus-r, Gus-s, Gus-t, Gus-u, Gut, asd, beta-glucuronidase, adipose storage deficiency
Species: mouse
Products:     Gusb

Top Publications

  1. Birkenmeier E, Davisson M, Beamer W, Ganschow R, Vogler C, Gwynn B, et al. Murine mucopolysaccharidosis type VII. Characterization of a mouse with beta-glucuronidase deficiency. J Clin Invest. 1989;83:1258-66 pubmed
    ..showed that the mutation is inherited as an autosomal recessive that maps to the beta-glucuronidase gene complex, [Gus], on the distal end of chromosome 5...
  2. Woloszynek J, Roberts M, Coleman T, Vogler C, Sly W, Semenkovich C, et al. Numerous transcriptional alterations in liver persist after short-term enzyme-replacement therapy in a murine model of mucopolysaccharidosis type VII. Biochem J. 2004;379:461-9 pubmed
    ..Malnutrition may account for the pronounced adipose storage deficiency observed in this animal...
  3. Birkenmeier E, Barker J, Vogler C, Kyle J, Sly W, Gwynn B, et al. Increased life span and correction of metabolic defects in murine mucopolysaccharidosis type VII after syngeneic bone marrow transplantation. Blood. 1991;78:3081-92 pubmed
    ..Life span in BMT-treated animals was increased approximately three-fold, approaching that seen in normal mice after BMT. These results support the position that BMT has a place in the therapeutic regimen for MPS VII. ..
  4. Snyder E, Taylor R, Wolfe J. Neural progenitor cell engraftment corrects lysosomal storage throughout the MPS VII mouse brain. Nature. 1995;374:367-70 pubmed
    ..beta-Glucuronidase activity was expressed along the entire neuraxis, resulting in widespread correction of lysosomal storage in neurons and glia in affected mice. ..
  5. Casal M, Wolfe J. Variant clinical course of mucopolysaccharidosis type VII in two groups of mice carrying the same mutation. Lab Invest. 1998;78:1575-81 pubmed
    ..The disease has been well-characterized in B6.C-H-2bml/ByBir-gus(mps/mps) mice, which have proven to be an excellent model for the study of treatment modalities of storage diseases...
  6. Vogler C, Levy B, Galvin N, Sands M, Birkenmeier E, Sly W, et al. A novel model of murine mucopolysaccharidosis type VII due to an intracisternal a particle element transposition into the beta-glucuronidase gene: clinical and pathologic findings. Pediatr Res. 2001;49:342-8 pubmed
    ..Affected gus(mps2J)/gus(mps2J) mice are deficient in beta-glucuronidase because of insertion of an intracisternal A particle ..
  7. Patarca R, Freeman G, Singh R, Wei F, Durfee T, Blattner F, et al. Structural and functional studies of the early T lymphocyte activation 1 (Eta-1) gene. Definition of a novel T cell-dependent response associated with genetic resistance to bacterial infection. J Exp Med. 1989;170:145-61 pubmed
    ..Eta-1 gene expression appears to be part of a surprisingly rapid T cell-dependent response to bacterial infection that may precede classical forms of T cell-dependent immunity. ..
  8. Zha H, Remmers E, Szpirer C, Szpirer J, Zhang H, Kozak C, et al. The epimorphin gene is highly conserved among humans, mice, and rats and maps to human chromosome 7, mouse chromosome 5, and rat chromosome 12. Genomics. 1996;37:386-9 pubmed
    ..The mouse and human homologs of this gene were localized on mouse chromosome 5 and human chromosome 7 by linkage analysis and chromosomal in situ hybridization, respectively. ..
  9. Tomatsu S, Orii K, Vogler C, Nakayama J, Levy B, Grubb J, et al. Mouse model of N-acetylgalactosamine-6-sulfate sulfatase deficiency (Galns-/-) produced by targeted disruption of the gene defective in Morquio A disease. Hum Mol Genet. 2003;12:3349-58 pubmed
    ..The complete absence of GALNS in mutant mice makes them useful for studies of pharmacokinetics and tissue targeting of recombinant GALNS designed for enzyme replacement. ..

More Information

Publications102 found, 100 shown here

  1. Kozak C, Rowe W. Genetic mapping of the ecotropic murine leukemia virus-inducing locus of BALB/c mouse to chromosome 5. Science. 1979;204:69-71 pubmed
    ..BALB/c mice, was mapped to chromosome 5, 23 units from the locus for phosphoglucomutase-1, with gene order Cv-Pgm-1-Gus. This low-efficiency inducibility locus is therefore not allelic with the chromosome 7 loci previously described ..
  2. Hudec P, Sonnenfeld P. Lysosomal and microsomal glucuronidase: genetic variant alters electrophoretic mobility of both hydrolases. Science. 1974;185:442-4 pubmed
    ..The difference is inherited as a single gene mapping at the position of the structural gene on chromosome 5. This confirms that beta-glucuronidase at both intracellular sites is coded by the same structural gene. ..
  3. Watson G, Davey R, Labarca C, Paigen K. Genetic determination of kinetic parameters in beta-glucuronidase induction by androgen. J Biol Chem. 1981;256:3005-11 pubmed
    A regulatory locus, Gus-r, determines the rate and extent of androgen inducibility of beta-glucuronidase in mouse kidney epithelial cells...
  4. Tamai Y, Nakajima R, Ishikawa T, Takaku K, Seldin M, Taketo M. Colonic hamartoma development by anomalous duplication in Cdx2 knockout mice. Cancer Res. 1999;59:2965-70 pubmed
    ..the heterozygotes were viable and fertile and expressed lacZ in the caudal region in early embryos and in the gut tissues in adults...
  5. Casal M, Wolfe J. Mucopolysaccharidosis type VII in the developing mouse fetus. Pediatr Res. 2000;47:750-6 pubmed
    ..5 d gestational age and in brains until birth. The results suggest that treatment of affected mice performed at 13.5 d gestational age may be effective in forestalling disease manifestations. ..
  6. Hofling A, Vogler C, Creer M, Sands M. Engraftment of human CD34+ cells leads to widespread distribution of donor-derived cells and correction of tissue pathology in a novel murine xenotransplantation model of lysosomal storage disease. Blood. 2003;101:2054-63 pubmed
    ..The beta-glucuronidase (GUSB) mutation of the mucopolysaccharidosis type VII (MPSVII) mouse was backcrossed onto the nonobese diabetic/severe ..
  7. Li B, Sharpe E, Maupin A, Teleron A, Pyle A, Carmeliet P, et al. VEGF and PlGF promote adult vasculogenesis by enhancing EPC recruitment and vessel formation at the site of tumor neovascularization. FASEB J. 2006;20:1495-7 pubmed
    ..Our data suggest local VEGF and/or PlGF expression promote vasculogenesis; VEGF plays a role in EPC recruitment and subsequent formation of functional vessels. ..
  8. Kozak C, Albritton L, Cunningham J. Genetic mapping of a cloned sequence responsible for susceptibility to ecotropic murine leukemia viruses. J Virol. 1990;64:3119-21 pubmed
    ..This locus maps in the distal region of chromosome 5 and is thus not within the cluster of retrovirus-related genes near the centromere. ..
  9. Gonzalez F, Liu S, Kozak C, Nebert D. Decreased Hnf-1 gene expression in mice homozygous for a 1.2-centiMorgan deletion on chromosome 7. DNA Cell Biol. 1990;9:771-6 pubmed
    ..These data suggest that the deleted region of chromosome 7 contains a gene encoding a trans-acting factor which is upstream (epistatic) in the regulatory cascade that includes Hnf-1 gene expression. ..
  10. Wang B, Korfhagen T, Gallagher P, D Amore M, McNeish J, Potter S, et al. Overlapping transcriptional units on the same strand within the murine beta-glucuronidase gene complex. J Biol Chem. 1988;263:15841-4 pubmed
    ..On the same strand and within the first intron of the beta-glucuronidase structural gene, Gus-s, we observe an RNA polymerase II promoter motif...
  11. Catterall J, Leary S. Detection of early changes in androgen-induced mouse renal beta-glucuronidase messenger ribonucleic acid using cloned complementary deoxyribonucleic acid. Biochemistry. 1983;22:6049-53 pubmed
    ..Treatment of female mice with a single dose of testosterone (10 mg) revealed that beta-glucuronidase mRNA concentration begins to increase between 12 and 24 h after hormone administration. ..
  12. Stein C, Ghodsi A, Derksen T, Davidson B. Systemic and central nervous system correction of lysosomal storage in mucopolysaccharidosis type VII mice. J Virol. 1999;73:3424-9 pubmed
    ..These results indicate that prolonged enzyme expression from transgenes delivered to deficient liver and brain can mediate pervasive correction and illustrate the potential for gene therapy of MPS and other lysosomal storage diseases. ..
  13. Caetano A, Shiue Y, Lyons L, O Brien S, Laughlin T, Bowling A, et al. A comparative gene map of the horse (Equus caballus). Genome Res. 1999;9:1239-49 pubmed
    ..The equine type I markers developed in this study provide an important resource for the future development of the horse linkage and physical genome maps. ..
  14. Groves M, Rosenstreich D, Taylor B, Osterman J. Host defenses in experimental scrub typhus: mapping the gene that controls natural resistance in mice. J Immunol. 1980;125:1395-9 pubmed
    ..Analyis of th C57BL/6JTy-le congenic strain indicates that Ric is proximal to rd on Chromosome 5. If so, the correct gene order is Pgm-1-W-Ric-rd-Gus.
  15. Welch C, Xia Y, Gu L, Machleder D, Mehrabian M, Wen P, et al. Srb1 maps to mouse chromosome 5 in a region harboring putative QTLs for plasma lipoprotein levels. Mamm Genome. 1997;8:942-4 pubmed
  16. Skorupa A, Fisher K, Wilson J, Parente M, Wolfe J. Sustained production of beta-glucuronidase from localized sites after AAV vector gene transfer results in widespread distribution of enzyme and reversal of lysosomal storage lesions in a large volume of brain in mucopolysaccharidosis VII mice. Exp Neurol. 1999;160:17-27 pubmed
  17. Ghodsi A, Stein C, Derksen T, Martins I, Anderson R, Davidson B. Systemic hyperosmolality improves beta-glucuronidase distribution and pathology in murine MPS VII brain following intraventricular gene transfer. Exp Neurol. 1999;160:109-16 pubmed
    ..Our findings have positive therapeutic implications for the treatment of CNS disorders with gene transfer vectors and recombinant proteins. ..
  18. Majumder M, Xin X, Liu L, Tutunea Fatan E, Rodriguez Torres M, Vincent K, et al. COX-2 Induces Breast Cancer Stem Cells via EP4/PI3K/AKT/NOTCH/WNT Axis. Stem Cells. 2016;34:2290-305 pubmed publisher
    ..MCF-7-COX-2 cells also showed increased lung colonization in NOD/SCID/GUSB-null mice, an effect reversed with EP4-knockdown or EP4A treatment of the MCF-7-COX-2 cells...
  19. Swank R, Paigen K, Ganschow R. Genetic control of glucuronidase induction in mice. J Mol Biol. 1973;81:225-43 pubmed
  20. Rosnet O, Stephenson D, Mattei M, Marchetto S, Shibuya M, Chapman V, et al. Close physical linkage of the FLT1 and FLT3 genes on chromosome 13 in man and chromosome 5 in mouse. Oncogene. 1993;8:173-9 pubmed
    ..The physical linkage is also evidenced in mouse, where the two genes appear to lie within a 350 kb Mlu I fragment, on mouse chromosome 5. ..
  21. Bramwell K, Mock K, Ma Y, Weis J, Teuscher C, Weis J. β-Glucuronidase, a Regulator of Lyme Arthritis Severity, Modulates Lysosomal Trafficking and MMP-9 Secretion in Response to Inflammatory Stimuli. J Immunol. 2015;195:1647-56 pubmed publisher
    The lysosomal enzyme β-glucuronidase (Gusb) is a key regulator of Lyme-associated and K/B×N-induced arthritis severity...
  22. Lunsford R, Jenkins N, Kozak C, Liang L, Silan C, Copeland N, et al. Genomic mapping of murine Zp-2 and Zp-3, two oocyte-specific loci encoding zona pellucida proteins. Genomics. 1990;6:184-7 pubmed
    ..3 +/- 3.2 cM distal to Tyr, and Zp-3 was located on chromosome 5, 9.2 +/- 2.9 cM distal to Gus.
  23. Danciger M, Bowes C, Kozak C, LaVail M, Farber D. Fine mapping of a putative rd cDNA and its co-segregation with rd expression. Invest Ophthalmol Vis Sci. 1990;31:1427-32 pubmed
    ..gene for this disease has been mapped to mouse chromosome 5 between the well-defined (anchor) genes Afp and Gus. We recently cloned a putative rd cDNA, zr...
  24. Daly T, Vogler C, Levy B, Haskins M, Sands M. Neonatal gene transfer leads to widespread correction of pathology in a murine model of lysosomal storage disease. Proc Natl Acad Sci U S A. 1999;96:2296-300 pubmed
    ..4 x 10(6) infectious units of recombinant adeno-associated virus encoding the human beta-glucuronidase (GUSB) cDNA...
  25. Freeman B, Roberts M, Vogler C, Nicholes A, Hofling A, Sands M. Behavior and therapeutic efficacy of beta-glucuronidase-positive mononuclear phagocytes in a murine model of mucopolysaccharidosis type VII. Blood. 1999;94:2142-50 pubmed
    ..These data suggest that macrophages play a significant role in the therapeutic efficacy of BMT for lysosomal storage diseases and may have implications for treatments such as gene therapy. ..
  26. Womack J, Hawes N, Soares E, Roderick T. Mitochondrial malate dehydrogenase (Mor-1) in the mouse: linkage to chromosome 5 markers. Biochem Genet. 1975;13:519-25 pubmed
    ..We have located this locus 11.0 +/- 2.9 cM from the beta-glucuronidase structural gene, Gus, on chromosome 5. The gene order is Hm-Pgm-1-rd-bf-Gus-Mor-1...
  27. Moore K, Paigen K. Genome organization and polymorphism of the murine beta-glucuronidase region. Genomics. 1988;2:25-31 pubmed
    ..A BamHI site is missing from the CS and CL haplotypes that share regulatory properties, and a 0.2-kb insertion is consistently present in haplotypes showing increased response to induction by androgens in kidney. ..
  28. Stephenson D, Lee K, Nagle D, Yen C, Morrow A, Miller D, et al. Mouse rump-white mutation associated with an inversion of chromosome 5. Mamm Genome. 1994;5:342-8 pubmed
    ..Taken together, both the genetic and physical mapping data establish that the Rw mutation is associated with an inversion involving loci in the proximal region of Chromosome 5. ..
  29. Donsante A, Vogler C, Muzyczka N, Crawford J, Barker J, Flotte T, et al. Observed incidence of tumorigenesis in long-term rodent studies of rAAV vectors. Gene Ther. 2001;8:1343-6 pubmed
    ..In an attempt to ascertain whether mouse strain or GUSB expression confers susceptibility to tumor formation, we histopathologically examined untreated normal mice of the ..
  30. Meng X, Shen J, Kawagoe S, Ohashi T, Brady R, Eto Y. Induced pluripotent stem cells derived from mouse models of lysosomal storage disorders. Proc Natl Acad Sci U S A. 2010;107:7886-91 pubmed publisher
    ..Thus, our study demonstrates the unique promise of iPS cells for studying the pathogenesis and treatment of LSDs. ..
  31. Harper J, Mould A, Andrews R, Bikoff E, Robertson E. The transcriptional repressor Blimp1/Prdm1 regulates postnatal reprogramming of intestinal enterocytes. Proc Natl Acad Sci U S A. 2011;108:10585-90 pubmed publisher
    ..In contrast, those required for processing maternal milk were markedly reduced. Thus, we conclude Blimp1 governs the developmental switch responsible for postnatal intestinal maturation. ..
  32. Pittler S, Lee A, Altherr M, Howard T, Seldin M, Hurwitz R, et al. Primary structure and chromosomal localization of human and mouse rod photoreceptor cGMP-gated cation channel. J Biol Chem. 1992;267:6257-62 pubmed
    ..The mouse channel gene locus (Cncg) was mapped by interspecific backcross haplotype analysis 0.9 centimorgan proximal of the Kit locus on chromosome 5. ..
  33. Wydner K, Sechler J, Boyd C, Passmore H. Use of an intron polymorphism to localize the tropoelastin gene to mouse chromosome 5 in a region of linkage conservation with human chromosome 7. Genomics. 1994;23:125-31 pubmed
    ..The linkage and genetic distances between Eln and the closest molecular markers used in this study are centromere-D5Mit95, D5Mit96-6.7 cM-Gus, Eln-4.0 cM-Zp3-telomere.
  34. Moullier P, Bohl D, Heard J, Danos O. Correction of lysosomal storage in the liver and spleen of MPS VII mice by implantation of genetically modified skin fibroblasts. Nat Genet. 1993;4:154-9 pubmed
    ..A complete disappearance of the lysosomal storage lesions was observed in their liver and spleen. ..
  35. Ohashi T, Watabe K, Uehara K, Sly W, Vogler C, Eto Y. Adenovirus-mediated gene transfer and expression of human beta-glucuronidase gene in the liver, spleen, and central nervous system in mucopolysaccharidosis type VII mice. Proc Natl Acad Sci U S A. 1997;94:1287-92 pubmed
    ..These results suggest that adenovirus-mediated gene delivery might improve the central nervous system pathology of mucopolysaccharidosis in addition to correcting visceral pathology. ..
  36. Hahm S, Mizuno T, Wu T, Wisor J, Priest C, Kozak C, et al. Targeted deletion of the Vgf gene indicates that the encoded secretory peptide precursor plays a novel role in the regulation of energy balance. Neuron. 1999;23:537-48 pubmed
  37. Uslu V, Petretich M, Ruf S, Langenfeld K, Fonseca N, Marioni J, et al. Long-range enhancers regulating Myc expression are required for normal facial morphogenesis. Nat Genet. 2014;46:753-8 pubmed publisher
    ..This dual molecular etiology may account for the prominent influence of variants in the 8q24 region on human facial dysmorphologies. ..
  38. Warden C, Krisans S, Purcell Huynh D, Leete L, Daluiski A, Diep A, et al. Mouse cellular nucleic acid binding proteins: a highly conserved family identified by genetic mapping and sequencing. Genomics. 1994;24:14-9 pubmed
    ..These data suggest that CNBP is a member of a highly conserved family of cytosolic proteins that may be encoded by multiple dispersed genes. ..
  39. Derrick Roberts A, Panir K, Pyragius C, Zarrinkalam K, Atkins G, Byers S. Reversal of established bone pathology in MPS VII mice following lentiviral-mediated gene therapy. Mol Genet Metab. 2016;119:249-257 pubmed publisher
    ..Collectively, these results indicate that lentiviral-mediated gene therapy is effective in reversing established skeletal pathology in murine MPS VII. ..
  40. Paigen K. Acid hydrolases as models of genetic control. Annu Rev Genet. 1979;13:417-66 pubmed
  41. Hanley Hyde J, Mushinski J, Sadofsky M, Huppi K, Krall M, Kozak C, et al. Expression of murine cyclin B1 mRNAs and genetic mapping of related genomic sequences. Genomics. 1992;13:1018-30 pubmed
    ..Cycb1-rs6 (on chromosome 13) is discussed as the most likely candidate for an expressed structural gene locus. ..
  42. Su H, Kozak C, Veerhuis R, Lau Y, Wiberg U. Isolation of a phylogenetically conserved and testis-specific gene using a monoclonal antibody against the serological H-Y antigen. J Reprod Immunol. 1992;21:275-91 pubmed
    ..Analysis of an intersubspecies mouse backcross has assigned this gene to chromosome 5, between the loci Gus and Hnf-1.
  43. Bell D, Taguchi T, Jenkins N, Gilbert D, Copeland N, Gilks C, et al. Chromosomal localization of a gene, GF1, encoding a novel zinc finger protein reveals a new syntenic region between man and rodents. Cytogenet Cell Genet. 1995;70:263-7 pubmed
    ..Comparative mapping data presented here describes a new syntenic region between man and rodents. ..
  44. Bassel Duby R, Hernandez M, Yang Q, Rochelle J, Seldin M, Williams R. Myocyte nuclear factor, a novel winged-helix transcription factor under both developmental and neural regulation in striated myocytes. Mol Cell Biol. 1994;14:4596-605 pubmed
  46. Bevilacqua A, Erickson R, Hieber V. Antisense RNA inhibits endogenous gene expression in mouse preimplantation embryos: lack of double-stranded RNA "melting" activity. Proc Natl Acad Sci U S A. 1988;85:831-5 pubmed
    ..These results are in accord with our inability to detect RNA-duplex "melting" activity in 1-cell mouse embryos. We suggest that it may be possible to analyze genetics of mammalian development by antisense techniques. ..
  47. Watson G, Felder M, Rabinow L, Moore K, Labarca C, Tietze C, et al. Properties of rat and mouse beta-glucuronidase mRNA and cDNA, including evidence for sequence polymorphism and genetic regulation of mRNA levels. Gene. 1985;36:15-25 pubmed
    ..Genetic variation in the extent of induction affected either the level of beta G mRNA or its efficiency of translation depending on the strain of mice tested. ..
  48. Faust J, Rodman J, Daniel P, Dice J, Bronson R. Two related proteolipids and dolichol-linked oligosaccharides accumulate in motor neuron degeneration mice (mnd/mnd), a model for neuronal ceroid lipofuscinosis. J Biol Chem. 1994;269:10150-5 pubmed
    ..The accumulation of the vacuolar subunit c in this model may prove to be a marker for distinguishing different forms of NCLs. ..
  49. Urosevic N, Silvia O, Sangster M, Mansfield J, Hodgetts S, Shellam G. Development and characterization of new flavivirus-resistant mouse strains bearing Flv(r)-like and Flv(mr) alleles from wild or wild-derived mice. J Gen Virol. 1999;80 ( Pt 4):897-906 pubmed
  50. Smith J, Wadleigh D, Xia Y, Mar R, Herschman H, Lusis A. Cloning and genomic localization of the murine LPS-induced CXC chemokine (LIX) gene, Scyb5. Immunogenetics. 2002;54:599-603 pubmed
    ..The sequence and mapping data suggest that the human ENA78-PBP-PF4 and GCP2- psi PBP-PF4V1 loci arose from an evolutionarily recent duplication of an ancestral locus related to the murine Lix-Pbp-Pf4 locus. ..
  51. Paigen K, Swank R, Tomino S, Ganschow R. The molecular genetics of mammalian glucuronidase. J Cell Physiol. 1975;85:379-92 pubmed
    ..Regulatory and temporal sites appear to act in a cis fashion to control the amount of enzyme synthesized from the adjacent structural allele on the same chromosome. ..
  52. Wolfe J, Deshmane S, Fraser N. Herpesvirus vector gene transfer and expression of beta-glucuronidase in the central nervous system of MPS VII mice. Nat Genet. 1992;1:379-84 pubmed
  53. Lovell D, Erickson R. Genetic variation in the shape of the mouse mandible and its relationship to glucocorticoid-induced cleft palate analyzed by using recombinant inbred lines. Genetics. 1986;113:755-64 pubmed
    ..There was no evidence that the variability in steroid-induced cleft palate incidence in the BXA;AXB RI lines is related to the variation in adult mandible shape as detected in this study. ..
  54. Niermann G, Watson G. Growth hormone and insulin-like growth factor-I enhance beta-glucuronidase gene activation by androgen in mouse kidney. Mol Cell Endocrinol. 1999;153:47-55 pubmed
    Beta-glucuronidase (GUS) is a lysosomal enzyme that, in mouse kidney, is subject to control by multiple hormones: androgen, which increases GUS transcription; estrogen, which antagonizes androgen-mediated stimulation of GUS; and growth ..
  55. Hill R, Jones P, Rees A, Sime C, Justice M, Copeland N, et al. A new family of mouse homeo box-containing genes: molecular structure, chromosomal location, and developmental expression of Hox-7.1. Genes Dev. 1989;3:26-37 pubmed
    ..Later, this gene is expressed in regions of the face that are derived from neural crest and in the interdigital mesenchymal tissues in both the fore- and hindlimbs. ..
  56. Boonen M, Vogel P, Platt K, Dahms N, Kornfeld S. Mice lacking mannose 6-phosphate uncovering enzyme activity have a milder phenotype than mice deficient for N-acetylglucosamine-1-phosphotransferase activity. Mol Biol Cell. 2009;20:4381-9 pubmed publisher
  57. Schreiber R, Taschler U, Wolinski H, Seper A, Tamegger S, Graf M, et al. Esterase 22 and beta-glucuronidase hydrolyze retinoids in mouse liver. J Lipid Res. 2009;50:2514-23 pubmed publisher
    ..Es22 was previously shown to form a complex with beta-glucuronidase (Gus). Our studies revealed that Gus colocalizes with Es22 at the ER but does not affect its RE hydrolase activity...
  58. Seldin M, Martinez L, Howard T, Naylor S, Sakaguchi A. Localization of mouse melanoma growth stimulatory activity gene (Mgsa) between Afp and Gus on chromosome 5 using interspecific backcross mice. Cytogenet Cell Genet. 1990;54:68-70 pubmed
    ..A restriction fragment length variant of Mgsa was followed in interspecific backcross mic Mgsa was localized between Afp and Gus on mouse chromoson 5, distal to known loci affecting skin pigmentation.
  59. Zhen L, Rusiniak M, Swank R. The beta-glucuronidase propeptide contains a serpin-related octamer necessary for complex formation with egasyn esterase and for retention within the endoplasmic reticulum. J Biol Chem. 1995;270:11912-20 pubmed
    ..These and related results demonstrate that complex formation with esterases/proteinases within the ER is important in the subcellular targeting and/or processing of certain proteins. ..
  60. Hulsebos T, Jenkins N, Gilbert D, Copeland N. The beta crystallin genes on human chromosome 22 define a new region of homology with mouse chromosome 5. Genomics. 1995;25:574-6 pubmed
    ..e., Crybb2, Crybb3, and Cryba4) to the central region of mouse chromosome 5. The homologue of human CRYBB2P1 is absent in mouse. These assignments define a new region of homology in human and mouse. ..
  61. Poorthuis B, Romme A, Willemsen R, Wagemaker G. Bone marrow transplantation has a significant effect on enzyme levels and storage of glycosaminoglycans in tissues and in isolated hepatocytes of mucopolysaccharidosis type VII mice. Pediatr Res. 1994;36:187-93 pubmed
    ..The subcellular localization of beta-glucuronidase was investigated in liver parenchymal cells of mice that underwent transplantation.(ABSTRACT TRUNCATED AT 250 WORDS) ..
  62. Clegg C, Koeiman N, Jenkins N, Gilbert D, Copeland N, Neubauer M. Structural features of the murine gene encoding the RI beta subunit of cAMP-dependent protein kinase. Mol Cell Neurosci. 1994;5:153-64 pubmed
    ..The abundance and configuration of transcription factor binding sites within the promoter region of RI beta suggests that this gene is subject to complex modes of regulation in neurons. ..
  63. Barker J, Deveau S, Lessard M, Hamblen N, Vogler C, Levy B. In utero fetal liver cell transplantation without toxic irradiation alleviates lysosomal storage in mice with mucopolysaccharidosis type VII. Blood Cells Mol Dis. 2001;27:861-73 pubmed
    ..In MPS VII mice, normal donor hematopoietic cells secrete the missing enzyme beta-glucuronidase (GUSB) that reverses disease manifestations...
  64. Baldo G, Wu S, Howe R, Ramamoothy M, Knutsen R, Fang J, et al. Pathogenesis of aortic dilatation in mucopolysaccharidosis VII mice may involve complement activation. Mol Genet Metab. 2011;104:608-19 pubmed publisher
    ..Complement may be activated by the GAGs that accumulate, and may play a role in signal transduction pathways that upregulate elastases. ..
  65. Sands M, Birkenmeier E. A single-base-pair deletion in the beta-glucuronidase gene accounts for the phenotype of murine mucopolysaccharidosis type VII. Proc Natl Acad Sci U S A. 1993;90:6567-71 pubmed
    Murine mucopolysaccharidosis type VII is a heritable disease caused by a spontaneous mutation, gus(mps), closely linked to the beta-glucuronidase structural gene on chromosome 5...
  66. Ramagli L, Womack J, Rodriguez L. Genetic analysis of nonhistone chromosomal protein inheritance in recombinant inbred mouse strains using two-dimensional electrophoresis. Biochem Genet. 1990;28:123-36 pubmed
    ..These 16 NHCP genetic marker inheritance differences detected in RIs add to the 23 previously established genetic marker differences between the progenitors. ..
  67. Okazaki Y, Hirose K, Hirotsune S, Okuizumi H, Sasaki N, Ohsumi T, et al. Direct detection and isolation of restriction landmark genomic scanning (RLGS) spot DNA markers tightly linked to a specific trait by using the RLGS spot-bombing method. Proc Natl Acad Sci U S A. 1995;92:5610-4 pubmed
    ..We identified 33 variant markers out of 10,565 total loci in a 4.2-centimorgan (cM) interval surrounding the Gus locus in 4 days of laboratory work...
  68. Asadi F, Dimaculangan D, Berger F. Androgen regulation of gene expression in primary epithelial cells of the mouse kidney. Endocrinology. 1994;134:1179-87 pubmed
  69. Zhou D, Cantu C, Sagiv Y, Schrantz N, Kulkarni A, Qi X, et al. Editing of CD1d-bound lipid antigens by endosomal lipid transfer proteins. Science. 2004;303:523-7 pubmed
    ..LTPs constitute a previously unknown link between lipid metabolism and immunity and are likely to exert a profound influence on the repertoire of self, tumor, and microbial lipid antigens. ..
  70. Xing E, Wu S, Ponder K. The effect of Tlr4 and/or C3 deficiency and of neonatal gene therapy on skeletal disease in mucopolysaccharidosis VII mice. Mol Genet Metab. 2015;114:209-16 pubmed publisher
    ..MPS) VII is a lysosomal storage disorder caused by the deficiency of the enzyme β-glucuronidase (Gusb(-/-)) and results in glycosaminoglycan (GAG) accumulation...
  71. Schlagel C, Ahmed A. Evidence for genetic control of microwave-induced augmentation of complement receptor-bearing B lymphocytes. J Immunol. 1982;129:1530-3 pubmed
    ..The C57BL/6JTy-le strain remained nonresponsive. This places the essential regulatory gene to the right of the PgM-1 locus and to the left of the rd locus on chromosome 5. ..
  72. Simonaro C, Ge Y, Eliyahu E, He X, Jepsen K, Schuchman E. Involvement of the Toll-like receptor 4 pathway and use of TNF-alpha antagonists for treatment of the mucopolysaccharidoses. Proc Natl Acad Sci U S A. 2010;107:222-7 pubmed publisher
    ..These studies revealed the important role of TLR4 signaling in MPS bone and joint disease and suggest that targeting TNF-alpha may have positive therapeutic effects. ..
  73. Parente M, Rozen R, Cearley C, Wolfe J. Dysregulation of gene expression in a lysosomal storage disease varies between brain regions implicating unexpected mechanisms of neuropathology. PLoS ONE. 2012;7:e32419 pubmed publisher
    ..In addition, the variation in gene dysregulation between brain regions indicates that different neuropathologic mechanisms may predominate within different regions of a diseased brain caused by a single gene mutation. ..
  74. McCubrey J, Risser R. Allelism and linkage studies of murine leukemia virus activation genes in low leukemic strains of mice. J Exp Med. 1982;155:1233-8 pubmed
    ..We also show that the Inc-1 locus of BALB/c mice is located on chromosome 5, 24 cM from Pgm-1 and 43 cM from Gus. Kozak and Rowe (6,8) and Ihle and co-workers (3) have shown that the ecotropic virus-inducing genes in BALB/c and ..
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    ..The third locus is unmapped. This study demonstrates that cloned repeats from a repetitive DNA library are a potential source of genetic markers. ..
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    ..type VII (MPS VII) is a heritable lysosomal storage disease caused by a deficiency in beta-glucuronidase (GUSB) activity, leading to progressive accumulation of undegraded glycosaminoglycans in many tissues...
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    ..5 to 4.5 d. These studies show that recombinant beta-glucuronidase administered to newborn mice reaches the sites of clinically important storage in murine mucopolysaccharidosis VII. ..
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    ..These data demonstrate the temporal pattern of retinal dysfunction in the MPS VII mouse and indicate that BMT can reduce the lysosomal storage and improve retinal function. ..