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Index > Protein center > GUSB(Gene name) > Human
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  • GUSB (Gene name),
  • Beta-glucuronidase (Protein name ),  BGLR_HUMAN from NCBI database.
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  • General Annotation
  • |
  • Antigen Annotation
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  • 3D
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  • Predicted Eptitope
  • |
  • Vaild Sequence
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  • Related Databases
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  • Gene name:
    GUSB;
    Protein name:
    Beta-glucuronidase;
    Alternative:
    Beta-G1;
    Organism:
    Human (Homo sapiens). 
    General Annotation
    Sub Unit:
    Homotetramer.
    Function:
    Plays an important role in the degradation of dermatan and keratan sulfates.
    Subcellular Location:
    Lysosome
    Protein Attributes:
    Sequence length:
    651
    Sequence:
    50:
    MARGSAVAWA | ALGPLLWGCA | LGLQGGMLYP | QESPSRECKE | LDGLWSFRAD | 
    100:
    FSDNRRRGFE | EQWYRRPLWE | SGPTVDMPVP | SSFNDISQDW | RLRHFVGWVW | 
    150:
    YEREVILPER | WTQDLRTRVV | LRIGSAHSYA | IVWVNGVDTL | EHEGGYLPFE | 
    200:
    ADISNLVQVG | PLPSRLRITI | AINNTLTPTT | LPPGTIQYLT | DTSKYPKGYF | 
    250:
    VQNTYFDFFN | YAGLQRSVLL | YTTPTTYIDD | ITVTTSVEQD | SGLVNYQISV | 
    300:
    KGSNLFKLEV | RLLDAENKVV | ANGTGTQGQL | KVPGVSLWWP | YLMHERPAYL | 
    350:
    YSLEVQLTAQ | TSLGPVSDFY | TLPVGIRTVA | VTKSQFLING | KPFYFHGVNK | 
    400:
    HEDADIRGKG | FDWPLLVKDF | NLLRWLGANA | FRTSHYPYAE | EVMQMCDRYG | 
    450:
    IVVIDECPGV | GLALPQFFNN | VSLHHHMQVM | EEVVRRDKNH | PAVVMWSVAN | 
    500:
    EPASHLESAG | YYLKMVIAHT | KSLDPSRPVT | FVSNSNYAAD | KGAPYVDVIC | 
    550:
    LNSYYSWYHD | YGHLELIQLQ | LATQFENWYK | KYQKPIIQSE | YGAETIAGFH | 
    600:
    QDPPLMFTEE | YQKSLLEQYH | LGLDQKRRKY | VVGELIWNFA | DFMTEQSPTR | 
    650:
    VLGNKKGIFT | RQRQPKSAAF | LLRERYWKIA | NETRYPHSVA | KSQCLENSLF | 
    651:
    T
    3D Structure:
    N/A
    Predicted Eptitope:
    Please Sign in.
    EIAab Sequence  Vaild Sequence:
    Please Sign in.
    Related Databases
    Pfam:
    SMR:
    MIM:
    KEGG:
    UniGene:
    String:
    Uniprot:
     
    FOR
    ELISA Kit for Human Beta-glucuronidase
    Cat.:
    E0202h
    Price:
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    MSDS:
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    Packing:
    96T
    Range:
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    ELISA Kit for Human Beta-glucuronidase
    Cat.:
    E0202r
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    MSDS:
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    Packing:
    96T
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    ELISA Kit for Human Beta-glucuronidase
    Cat.:
    E0202m
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    MSDS:
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    Packing:
    96T
    Range:
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    ELISA Kit for Human Beta-glucuronidase
    Cat.:
    E0202d
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    MSDS:
    Please sign in first.
    Packing:
    96T
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    ELISA Kit for Human Beta-glucuronidase
    Cat.:
    E0202p
    Price:
    Please sign in first.
    MSDS:
    Please sign in first.
    Packing:
    96T
    CLIA Kit for Human Beta-glucuronidase
    Cat.:
    U0202m
    Price:
    Please sign in first.
    MSDS:
    Please sign in first.
    Packing:
    96T
    Range:
    Please sign in first.
    CLIA Kit for Human Beta-glucuronidase
    Cat.:
    U0202r
    Price:
    Please sign in first.
    MSDS:
    Please sign in first.
    Packing:
    96T
    Range:
    Please sign in first.
    CLIA Kit for Human Beta-glucuronidase
    Cat.:
    U0202p
    Price:
    Please sign in first.
    MSDS:
    Please sign in first.
    Packing:
    96T
    CLIA Kit for Human Beta-glucuronidase
    Cat.:
    U0202h
    Price:
    Please sign in first.
    MSDS:
    Please sign in first.
    Packing:
    96T
    CLIA Kit for Human Beta-glucuronidase
    Cat.:
    U0202d
    Price:
    Please sign in first.
    MSDS:
    Please sign in first.
    Packing:
    96T
    Polyclonal Antibody for Human Beta-glucuronidase
    Polyclonal Antibody for Human Beta-glucuronidase
    Polyclonal Antibody for Human Beta-glucuronidase
    Cat.:
    P0202Rb-h
    Price:
    Please sign in first.
    Packing:
    40ug/0.2ml
    Polyclonal Antibody for Human Beta-glucuronidase
    Cat.:
    P0202Rb-r
    Price:
    Please sign in first.
    Packing:
    40ug/0.2ml
    Polyclonal Antibody for Human Beta-glucuronidase
    Cat.:
    P0202Rb-m
    Price:
    Please sign in first.
    Packing:
    40ug/0.2ml
    Monoclonal Antibody for Human Beta-glucuronidase
    Monoclonal Antibody for Human Beta-glucuronidase
    Monoclonal Antibody for Human Beta-glucuronidase
    Monoclonal Antibody for Human Beta-glucuronidase
    Monoclonal Antibody for Human Beta-glucuronidase
    Protein for Human Beta-glucuronidase
    Protein for Human Beta-glucuronidase
    Protein for Human Beta-glucuronidase
    Protein for Human Beta-glucuronidase
    Protein for Human Beta-glucuronidase

    R&D Technical Data
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    Precision
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    Recovery
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    Linearity
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    References
    1. 1.
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1);VARIANT PRO-649;ALTERNATIVE SPLICING
      tissue: Placenta.
    2. 2.
      "Complete sequencing and characterization of 21,243 full-length human cDNAs."
      Ota T. , Suzuki Y. , Nishikawa T. , Otsuki T. , Sugiyama T. , Irie R. , Wakamatsu A. , Hayashi K. , Sato H. , Nagai K. , Kimura K. , Makita H. , Sekine M. , Obayashi M. , Nishi T. , Shibahara T. , Tanaka T. , Ishii S. , more...
      Nat. Genet.36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 3);VARIANT PRO-649
      tissue: Lung.
    3. 3.
      Totoki Y. , Toyoda A. , Takeda T. , Sakaki Y. , Tanaka A. , Yokoyama S.
      Submitted (2005-04) to the EMBL/GenBank/DDBJ databases
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1)
    4. 4.
      "The DNA sequence of human chromosome 7."
      Hillier L.W. , Fulton R.S. , Fulton L.A. , Graves T.A. , Pepin K.H. , Wagner-McPherson C. , Layman D. , Maas J. , Jaeger S. , Walker R. , Wylie K. , Sekhon M. , Becker M.C. , O'Laughlin M.D. , Schaller M.E. , Fewell G.A. , Delehaunty K.D. , Miner T.L. , more...
      Nature424:157-164(2003) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA]
    5. 6.
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA]
    6. 7.
      "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
      The MGC Project Team
      Genome Res.14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1)
      tissue: Colon.
    7. 8.
      "Analysis of the 5' flanking region of the human beta-glucuronidase gene."
      Shipley J.M. , Miller R.D. , Wu B.M. , Grubb J.H. , Christensen S.G. , Kyle J.W. , Sly W.S.
      Genomics10:1009-1018(1991) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] OF 1-70
    8. 9.
      "Characterization of the subunits and sugar moiety of human placental and leukemic beta-glucuronidase."
      Tanaka J. , Gasa S. , Sakurada K. , Miyazaki T. , Kasai M. , Makita A.
      Biol. Chem. Hoppe-Seyler373:57-62(1992) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: PROTEIN SEQUENCE OF 23-32 AND 160-175
      tissue: Placenta.
    9. 10.
      "Isolation and expression in Escherichia coli of a cDNA clone encoding human beta-glucuronidase."
      Guise K.S. , Korneluk R.G. , Waye J. , Lamhonwah A.-M. , Quan F. , Palmer R. , Ganschow R.E. , Sly W.S. , Gravel R.A.
      Gene34:105-110(1985) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 520-585
      tissue: Fibroblast.
    10. 11.
      "A novel inhibitor of beta-glucuronidase: L-aspartic acid."
      Kreamer B.L. , Siegel F.L. , Gourley G.R.
      Pediatr. Res.50:460-466(2001) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: INHIBITION BY L-ASPARTIC ACID
    11. 12.
      "Identification and quantification of N-linked glycoproteins using hydrazide chemistry, stable isotope labeling and mass spectrometry."
      Zhang H. , Li X.-J. , Martin D.B. , Aebersold R.
      Nat. Biotechnol.21:660-666(2003) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: GLYCOSYLATION AT ASN-272
    12. 13.
      "Human plasma N-glycoproteome analysis by immunoaffinity subtraction, hydrazide chemistry, and mass spectrometry."
      Liu T. , Qian W.-J. , Gritsenko M.A. , Camp D.G. II , Monroe M.E. , Moore R.J. , Smith R.D.
      J. Proteome Res.4:2070-2080(2005) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-272
      tissue: Plasma.
    13. 14.
      "Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry."
      Chen R. , Jiang X. , Sun D. , Han G. , Wang F. , Ye M. , Wang L. , Zou H.
      J. Proteome Res.8:651-661(2009) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-173; ASN-272 AND ASN-631
      tissue: Liver.
    14. 15.
      "Structure of human beta-glucuronidase reveals candidate lysosomal targeting and active-site motifs."
      Jain S. , Drendel W.B. , Chen Z.W. , Mathews F.S. , Sly W.S. , Grubb J.H.
      Nat. Struct. Biol.3:375-381(1996) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: X-RAY CRYSTALLOGRAPHY (2.6 ANGSTROMS)
    15. 16.
      "Mutations and polymorphisms in GUSB gene in mucopolysaccharidosis VII (Sly Syndrome)."
      Tomatsu S. , Montano A.M. , Dung V.C. , Grubb J.H. , Sly W.S.
      Hum. Mutat.30:511-519(2009) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: REVIEW ON VARIANTS
    16. 17.
      "Molecular analysis of a patient with hydrops fetalis caused by beta-glucuronidase deficiency, and evidence for additional pseudogenes."
      Vervoort R. , Lissens W. , Liebaers I.
      Hum. Mutat.2:443-445(1993) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANT MPS7 TRP-216
    17. 18.
      "Mutational studies in a patient with the hydrops fetalis form of mucopolysaccharidosis type VII."
      Wu B.M. , Sly W.S.
      Hum. Mutat.2:446-457(1993) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANTS MPS7 VAL-354 AND TRP-611
    18. 19.
      "Mucopolysaccharidosis type VII: characterization of mutations and molecular heterogeneity."
      Tomatsu S. , Fukuda S. , Sukegawa K. , Ikedo Y. , Yamada S. , Yamada Y. , Sasaki T. , Okamoto H. , Kuwahara T. , Yamaguchi S. , Kiman T. , Shintaku H. , Isshiki G. , Orii T.
      Am. J. Hum. Genet.48:89-96(1991) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANTS MPS7 CYS-382 AND VAL-619
    19. 20.
      "Mutational analysis of a patient with mucopolysaccharidosis type VII, and identification of pseudogenes."
      Shipley J.M. , Klinkenberg M. , Wu B.M. , Bachinsky D.R. , Grubb J.H. , Sly W.S.
      Am. J. Hum. Genet.52:517-526(1993) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANT MPS7 CYS-627
    20. 21.
      "Overexpression rescues the mutant phenotype of L176F mutation causing beta-glucuronidase deficiency mucopolysaccharidosis in two Mennonite siblings."
      Wu B.M. , Tomatsu S. , Fukuda S. , Sukegawa K. , Orii T. , Sly W.S.
      J. Biol. Chem.269:23681-23688(1994) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANT MPS7 PHE-176;VARIANT PRO-649
    21. 22.
      "A pseudodeficiency allele (D152N) of the human beta-glucuronidase gene."
      Vervoort R. , Islam M.R. , Sly W. , Chabas A. , Wevers R. , de Jong J. , Liebaers I. , Lissens W.
      Am. J. Hum. Genet.57:798-804(1995) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANT MPS7 PHE-176;VARIANT ASN-152;CHARACTERIZATION OF VARIANT ASN-152
    22. 23.
      "Four novel mutations in mucopolysaccharidosis type VII including a unique base substitution in exon 10 of the beta-glucuronidase gene that creates a novel 5'-splice site."
      Yamada S. , Tomatsu S. , Sly W.S. , Islam R. , Wenger D.A. , Fukuda S. , Sukegawa K. , Orii T.
      Hum. Mol. Genet.4:651-655(1995) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANTS MPS7 SER-148 AND CYS-495
    23. 24.
      "Molecular analysis of patients with beta-glucuronidase deficiency presenting as hydrops fetalis or as early mucopolysaccharidosis VII."
      Vervoort R. , Islam M.R. , Sly W.S. , Zabot M.T. , Kleijer W.J. , Chabas A. , Fensom A. , Young E.P. , Liebaers I. , Lissens W.
      Am. J. Hum. Genet.58:457-471(1996) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANTS MPS7 ARG-136; LYS-150; PHE-176; TRP-216; CYS-320; SER-320; TYR-351; CYS-374; CYS-382; HIS-382; PRO-435; TRP-477; CYS-508; ASP-572; ASN-606 AND CYS-627
    24. 25.
      "beta-Glucuronidase P408S, P415L mutations: evidence that both mutations combine to produce an MPS VII allele in certain Mexican patients."
      Islam M.R. , Vervoort R. , Lissens W. , Hoo J.J. , Valentino L.A. , Sly W.S.
      Hum. Genet.98:281-284(1996) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANTS MPS7 SER-408 AND LEU-415
    25. 26.
      "Molecular analysis of the beta-glucuronidase gene: novel mutations in mucopolysaccharidosis type VII and heterogeneity of the polyadenylation region."
      Vervoort R. , Buist N.R. , Kleijer W.J. , Wevers R. , Fryns J.P. , Liebaers I. , Lissens W.
      Hum. Genet.99:462-468(1997) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANT MPS7 PHE-52;CHARACTERIZATION OF VARIANT MPS7 PHE-52
    26. 27.
      "Low beta-glucuronidase enzyme activity and mutations in the human beta-glucuronidase gene in mild mucopolysaccharidosis type VII, pseudodeficiency and a heterozygote."
      Vervoort R. , Gitzelmann R. , Bosshard N. , Maire I. , Liebaers I. , Lissens W.
      Hum. Genet.102:69-78(1998) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANT ASN-152;VARIANTS MPS7 GLY-38 AND HIS-626
    27. 28.
      "Mucopolysaccharidosis VII: clinical, biochemical and molecular investigation of a Brazilian family."
      Schwartz I. , Silva L.R. , Leistner S. , Todeschini L.A. , Burin M.G. , Pina-Neto J.M. , Islam R.M. , Shah G.N. , Sly W.S. , Giugliani R.
      Clin. Genet.64:172-175(2003) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANT MPS7 PHE-176
    28. 29.
      "Mutational analysis in longest known survivor of mucopolysaccharidosis type VII."
      Storch S. , Wittenstein B. , Islam R. , Ullrich K. , Sly W.S. , Braulke T.
      Hum. Genet.112:190-194(2003) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANTS MPS7 ASN-350 AND LEU-577;CHARACTERIZATION OF VARIANT MPS7 ASN-350 LEU-577
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