Recombinant人beta glucuronidase (GUSB) protein (ab114652)

概述

  • 产品名称Recombinant人beta glucuronidase (GUSB) protein
  • 蛋白长度Full length protein

描述

  • 性质Recombinant
  • 来源Wheat germ
  • 氨基酸序列
    • AccessionP08236
    • 种属Human
    • 序列GLQGGMLYPQESPSRECKELDGLWSFRADFSDNRRRGFEEQWYRRPLWES GPTVDMPVPSSFNDISQDWRLRHFVGWVWYEREVILPERWTQDLRTRVVL RIGSAHSYAIVWVNGVDTLEHEGGYLPFEADISNLVQVGPLPSRLRITIA INNTLTPTTLPPGTIQYLTDTSKYPKGYFVQNTYFDFFNYAGLQRSVLLY TTPTTYIDDITVTTSVEQDSGLVNYQISVKGSNLFKLEVRLLDAENKVVA NGTGTQGQLKVPGVSLWWPYLMHERPAYLYSLEVQLTAQTSLGPVSDFYT LPVGIRTVAVTKSQFLINGKPFYFHGVNKHEDADIRGKGFDWPLLVKDFN LLRWLGANAFRTSHYPYAEEVMQMCDRYGIVVIDECPGVGLALPQFFNNV SLHHHMQVMEEVVRRDKNHPAVVMWSVANEPASHLESAGYYLKMVIAHTK SLDPSRPVTFVSNSNYAADKGAPYVDVICLNSYYSWYHDYGHLELIQLQL ATQFENWYKKYQKPIIQSEYGAETIAGFHQDPPLMFTEEYQKSLLEQYHL GLDQKRRKYVVGELIWNFADFMTEQSPTRVLGNKKGIFTRQRQPKSAAFL LRERYWKIANETRYPHSVAKSQCLENSLFT
    • 分子量95 kDa including tags
    • 氨基酸22 to 651

技术指标

Our Abpromise guarantee covers the use of ab114652 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • 应用

    ELISA

    SDS-PAGE

    Western blot

  • 形式Liquid
  • 补充说明H00002990-P01
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.3% Glutathione, 0.79% Tris HCl

常规信息

  • 别名
    • Ac2-223
    • asd
    • Beta G1
    • Beta glucuronidase
    • Beta-G1
    • Beta-glucuronidase
    • BG
    • BGLR
    • BGLR_HUMAN
    • FLJ39445
    • Glucuronidase beta
    • Gur
    • Gus
    • Gus-r
    • Gus-s
    • Gus-t
    • Gus-u
    • GUSB
    • Gut
    • MPS7
    see all
  • 功能Plays an important role in the degradation of dermatan and keratan sulfates.
  • 疾病相关Defects in GUSB are the cause of mucopolysaccharidosis type 7 (MPS7) [MIM:253220]; also known as Sly syndrome. MPS7 is an autosomal recessive lysosomal storage disease characterized by inability to degrade glucuronic acid-containing glycosaminoglycans. The phenotype is highly variable, ranging from severe lethal hydrops fetalis to mild forms with survival into adulthood. Most patients with the intermediate phenotype show hepatomegaly, skeletal anomalies, coarse facies, and variable degrees of mental impairment.
    Note=Mucopolysaccharidosis type 7 is associated with non-immune hydrops fetalis, a generalized edema of the fetus with fluid accumulation in the body cavities due to non-immune causes. Non-immune hydrops fetalis is not a diagnosis in itself but a symptom, a feature of many genetic disorders, and the end-stage of a wide variety of disorders.
  • 序列相似性Belongs to the glycosyl hydrolase 2 family.
  • 翻译后修饰N-linked glycosylated with 3 to 4 oligosaccharide chains.
  • 细胞定位Lysosome.
  • Information by UniProt

Recombinant Human beta glucuronidase (GUSB) protein 图像

  • 12.5% SDS-PAGE showing ab114652 at approximately 95.41kDa stained with Coomassie Blue.

Recombinant Human beta glucuronidase (GUSB) protein (ab114652)参考文献

ab114652 has not yet been referenced specifically in any publications.

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