重组人Fumarylacetoacetate hydrolase蛋白(ab152377)

概述

描述

  • 性质
    Recombinant
  • 来源
    Wheat germ
  • 氨基酸序列
    • Accession
    • 种属
      Human
    • 序列
      MSFIPVAEDSDFPIHNLPYGVFSTRGDPRPRIGVAIGDQILDLSIIKHLF TGPVLSKHQDVFNQPTLNSFMGLGQAAWKEARVFLQNLLSVSQARLRDDT ELRKCAFISQASATMHLPATIGDYTDFYSSRQHATNVGIMFRDKENALMP NWLHLPVGYHGRASSVVVSGTPIRRPMGQMKPDDSKPPVYGACKLLDMEL EMAFFVGPGNRLGEPIPISKAHEHIFGMVLMNDWSARDIQKWEYVPLGPF LGKSFGTTVSPWVVPMDALMPFAVPNPKQDPRPLPYLCHDEPYTFDINLS VNLKGEGMSQAATICKSNFKYMYWTMLQQLTHHSVNGCNLRPGDLLASGT ISGPEPENFGSMLELSWKGTKPIDLGNGQTRKFLLDGDEVIITGYCQGDG YRIGFGQCAGKVLPALLPS
    • 分子量
      73 kDa including tags
    • 氨基酸
      1 to 419

技术指标

Our Abpromise guarantee covers the use of ab152377 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

    Western blot

    SDS-PAGE

  • 形式
    Liquid
  • 补充说明
    Protein concentration is above or equal to 0.05 µg/µl.
  • 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: 0.31% Glutathione, 0.79% Tris HCl

常规信息

  • 别名
    • Beta diketonase
    • FAA
    • FAH
    • Fumarylacetoacetase
    • Fumarylacetoacetate
    • Fumarylacetoacetate hydrolase
    see all
  • 相关性
    Fumarylacetoacetate hydrolase is the last enzyme in the tyrosine catabolism pathway. FAH deficiency is associated with Type 1 hereditary tyrosinemia (HT). This is an autosomal recessive inborn error of metabolism that occurs in both an acute and a chronic form. Clinical characteristics of the acute form include hepatic failure and death in infancy, whereas children with the chronic form have renal tubular dysfunction and hypophosphatemic rickets, progressive liver disease with development of hepatocellular carcinoma. Dietary treatment with restriction of tyrosine and phenylalanine alleviates the rickets, but liver transplantation has so far been the only definite treatment.

图片

  • 12.5% SDS-PAGE analysis of ab152377 stained with Coomassie Blue.

文献

ab152377 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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