Recombinant Human beta glucuronidase (GUSB) protein (ab114652)

製品の概要

  • 製品名Recombinant Human beta glucuronidase (GUSB) protein
  • タンパク質長Full length protein

製品の詳細

  • 由来Recombinant
  • 由来Wheat germ
  • アミノ酸配列
    • アクセッション番号P08236
    • 生物種Human
    • 配列GLQGGMLYPQESPSRECKELDGLWSFRADFSDNRRRGFEEQWYRRPLWES GPTVDMPVPSSFNDISQDWRLRHFVGWVWYEREVILPERWTQDLRTRVVL RIGSAHSYAIVWVNGVDTLEHEGGYLPFEADISNLVQVGPLPSRLRITIA INNTLTPTTLPPGTIQYLTDTSKYPKGYFVQNTYFDFFNYAGLQRSVLLY TTPTTYIDDITVTTSVEQDSGLVNYQISVKGSNLFKLEVRLLDAENKVVA NGTGTQGQLKVPGVSLWWPYLMHERPAYLYSLEVQLTAQTSLGPVSDFYT LPVGIRTVAVTKSQFLINGKPFYFHGVNKHEDADIRGKGFDWPLLVKDFN LLRWLGANAFRTSHYPYAEEVMQMCDRYGIVVIDECPGVGLALPQFFNNV SLHHHMQVMEEVVRRDKNHPAVVMWSVANEPASHLESAGYYLKMVIAHTK SLDPSRPVTFVSNSNYAADKGAPYVDVICLNSYYSWYHDYGHLELIQLQL ATQFENWYKKYQKPIIQSEYGAETIAGFHQDPPLMFTEEYQKSLLEQYHL GLDQKRRKYVVGELIWNFADFMTEQSPTRVLGNKKGIFTRQRQPKSAAFL LRERYWKIANETRYPHSVAKSQCLENSLFT
    • 分子量95 kDa including tags
    • 領域22 to 651

特性

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.

  • アプリケーション

    ELISA

    SDS-PAGE

    Western blot

  • 製品の状態Liquid
  • 備考H00002990-P01
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

    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

関連情報

  • 別名
    • 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
  • 機能Plays an important role in the degradation of dermatan and keratan sulfates.
  • 関連疾患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.
  • 配列類似性Belongs to the glycosyl hydrolase 2 family.
  • 翻訳後修飾N-linked glycosylated with 3 to 4 oligosaccharide chains.
  • 細胞内局在Lysosome.
  • Information by UniProt

Recombinant Human beta glucuronidase (GUSB) protein 画像

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

Recombinant Human beta glucuronidase (GUSB) protein (ab114652) 使用論文

ab114652 has not yet been referenced specifically in any publications.

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