Recombinant人HEXB protein (ab114915)

概述

描述

  • 性质Recombinant
  • 来源Wheat germ
  • 氨基酸序列
    • AccessionP07686
    • 种属Human
    • 序列MELCGLGLPRPPMLLALLLATLLAAMLALLTQVALVVQVAEAARAPSVSA KPGPALWPLPLSVKMTPNLL HLAPENFYISHSPNSTAGPSCTLLEEAF RRYHGYIFGFYKWHHEPAEFQAKTQVQQLLVSITLQSECDAF PNISSD ESYTLLVKEPVAVLKANRVWGALRGLETFSQLVYQDSYGTFTINESTIID SPRFSHRGILIDTS RHYLPVKIILKTLDAMAFNKFNVLHWHIVDDQSF PYQSITFPELSNKGSYSLSHVYTPNDVRMVIEYARL RGIRVLPEFDTP GHTLSWGKGQKDLLTPCYSRQNKLDSFGPINPTLNTTYSFLTTFFKEISE VFPDQFIH LGGDEVEFKCWESNPKIQDFMRQKGFGTDFKKLESFYIQK VLDIIATINKGSIVWQEVFDDKAKLAPGTI VEVWKDSAYPEELSRVTA SGFPVILSAPWYLDLISYGQDWRKYYKVEPLDFGGTQKQKQLFIGGEACL WG EYVDATNLTPRLWPRASAVGERLWSSKDVRDMDDAYDRLTRHRCRM VERGIAAQPLYAGYCNHENM
    • 分子量87 kDa including tags
    • 氨基酸1 to 556

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技术指标

Our Abpromise guarantee covers the use of ab114915 in the following tested applications.

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

  • 应用

    Western blot

    SDS-PAGE

    ELISA

  • 形式Liquid
  • 补充说明Protein concentration is above or equal to 0.05 mg/ml. Best use within three months from the date of receipt of this protein.
  • 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
    • Beta hexosaminidase beta chain
    • Beta hexosaminidase subunit beta
    • Beta N acetylhexosaminidase
    • Beta-hexosaminidase subunit beta chain A
    • Beta-N-acetylhexosaminidase subunit beta
    • Cervical cancer proto oncogene 7 protein
    • Cervical cancer proto-oncogene 7 protein
    • ENC 1AS
    • Epididymis luminal protein 248
    • HCC 7
    • HCC-7
    • HCC7
    • HEL 248
    • HEX B
    • Hexb
    • HEXB_HUMAN
    • Hexosaminidase B
    • Hexosaminidase B (beta polypeptide)
    • Hexosaminidase subunit B
    • HexosaminidaseB
    • N acetyl beta glucosaminidase
    • N-acetyl-beta-glucosaminidase subunit beta
    see all
  • 功能Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues.
  • 疾病相关Defects in HEXB are the cause of GM2-gangliosidosis type 2 (GM2G2) [MIM:268800]; also known as Sandhoff disease. GM2-gangliosidosis is an autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. GM2G2 is clinically indistinguishable from GM2-gangliosidosis type 1, presenting startle reactions, early blindness, progressive motor and mental deterioration, macrocephaly and cherry-red spots on the macula.
  • 序列相似性Belongs to the glycosyl hydrolase 20 family.
  • 翻译后修饰N-linked glycans at Asn-142 and Asn-190 consist of Man(3)-GlcNAc(2) and Man(5 to 7)-GlcNAc(2), respectively.
    The beta-A and beta-B chains are produced by proteolytic processing of the precursor beta chain.
  • 细胞定位Lysosome.
  • Information by UniProt

Recombinant Human HEXB protein 图像

  • 12.5% SDS-PAGE showing ab114915 at approximately 87.23kDa.
    Stained with Coomassie Blue.

Recombinant Human HEXB protein (ab114915)参考文献

ab114915 has not yet been referenced specifically in any publications.

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