Recombinant人SGSH protein (ab116779)

概述

  • 产品名称
    Recombinant人SGSH protein
  • 蛋白长度
    Full length protein

描述

  • 性质
    Recombinant
  • 来源
    Wheat germ
  • 氨基酸序列
    • Accession
    • 种属
      Human
    • 序列
      MSCPVPACCALLLVLGLCRARPRNALLLLADDGGFESGAYNNSAIATPHL DALARRSLLFRNAFTSVSSCSPSRASLLTGLPQHQNGMYGLHQDVHHFNS FDKVRSLPLLLSQAGVRTGIIGKKHVGPETVYPFDFAYTEENGSVLQVGR NITRIKLLVRKFLQTQDDRPFFLYVAFHDPHRCGHSQPQYGTFCEKFGNG ESGMGRIPDWTPQAYDPLDVLVPYFVPNTPAARADLAAQYTTVGRMDQGV GLVLQELRDAGVLNDTLVIFTSDNGIPFPSGRTNLYWPGTAEPLLVSSPE HPKRWGQVSEAYVSLLDLTPTILDWFSIPYPSYAIFGSKTIHLTGRSLLP ALEAEPLWATVFGSQSHHEVTMSYPMRSVQHRHFRLVHNLNFKMPFPIDQ DFYVSPTFQDLLNRTTAGQPTGWYKDLRHYYYRARWELYDRSRDPHETQN LATDPRFAQLLEMLRDQLAKWQWETHDPWVCAPDGVLEEKLSPQCQPLHN EL
    • 分子量
      81 kDa including tags
    • 氨基酸
      1 to 502

技术指标

Our Abpromise guarantee covers the use of ab116779 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 used within three months from the date of receipt.
  • 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
    • Heparan sulfate sulfatase
    • Heparan sulphate sulphatase
    • HSS
    • MPS 3A
    • MPS3 A
    • MPS3A
    • Mucopolysaccharidosis type IIIA
    • N sulfoglucosamine sulfohydrolase
    • N sulfoglucosamine sulfohydrolase (sulfamidase)
    • N-sulphoglucosamine sulphohydrolase
    • SFMD
    • SGSH
    • SPHM_HUMAN
    • Sulfoglucosamine sulfamidase
    • Sulphamidase
    • Sulphoglucosamine sulphamidase
    see all
  • 疾病相关
    Defects in SGSH are the cause of mucopolysaccharidosis type 3A (MPS3A) [MIM:252900]; also known as Sanfilippo syndrome A. MPS3A is a severe form of mucopolysaccharidosis type 3, an autosomal recessive lysosomal storage disease due to impaired degradation of heparan sulfate. MPS3 is characterized by severe central nervous system degeneration, but only mild somatic disease. Onset of clinical features usually occurs between 2 and 6 years; severe neurologic degeneration occurs in most patients between 6 and 10 years of age, and death occurs typically during the second or third decade of life. MPS3A is characterized by earlier onset, rapid progression of symptoms and shorter survival.
  • 序列相似性
    Belongs to the sulfatase family.
  • 翻译后修饰
    The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity.
  • 细胞定位
    Lysosome.
  • Information by UniProt

图片

  • 12.5% SDS-PAGE showing ab116779 at approximately 81.29kDa.
    Stained with Coomassie Blue.

文献

ab116779 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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