重组人Lysosomal acid lipase蛋白(ab152501)

概述

  • 产品名称
    重组人Lysosomal acid lipase蛋白
  • 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
      MKMRFLGLVVCLVLWPLHSEGSGGKLTALDPETNMNVSEIISYWGFPSEE YLVETEDGYILCLNRIPHGRKNHSDKGPKPVVFLQHGLLADSSNWVTNLA NSSLGFILADAGFDVWMGNSRGNTWSRKHKTLSVSQDEFWAFSYDEMAKY DLPASINFILNKTGQEQVYYVGHSQGTTIGFIAFSQIPELAKRIKMFFAL GPVASVAFCTSPMAKLGRLPDHLIKDLFGDKEFLPQSAFLKWLGTHVCTH VILKELCGNLCFLLCGFNERNLNMSRVDVYTTHSPAGTSVQNMLHWSQAV KFQKFQAFDWGSSAKNYFHYNQSYPPTYNVKDMLVPTAVWSGGHDWLADV YDVNILLTQITNLVFHESIPEWEHLDFIWGLDAPWRLYNKIINLMRKYQ
    • 分子量
      70 kDa including tags
    • 氨基酸
      1 to 399
    • 标签
      GST tag N-Terminus

相关产品

技术指标

Our Abpromise guarantee covers the use of ab152501 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
    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
    • Acid cholesteryl ester hydrolase
    • CESD
    • cholesterol ester hydrolase
    • cholesterol ester storage disease
    • Cholesteryl esterase
    • Hydrolase deficiency
    • LAL
    • LAL deficiency cholesterol ester
    • LICH_HUMAN
    • lipA
    • LIPA deficiency
    • Lipase A
    • lipase A, lysosomal acid, cholesterol esterase
    • lysosomal acid lipase
    • lysosomal acid lipase deficiency
    • Lysosomal acid lipase/cholesteryl ester hydrolase
    • Sterol esterase
    see all
  • 功能
    Crucial for the intracellular hydrolysis of cholesteryl esters and triglycerides that have been internalized via receptor-mediated endocytosis of lipoprotein particles. Important in mediating the effect of LDL (low density lipoprotein) uptake on suppression of hydroxymethylglutaryl-CoA reductase and activation of endogenous cellular cholesteryl ester formation.
  • 疾病相关
    Defects in LIPA are the cause of Wolman disease (WOD) [MIM:278000]. WOD is a severe manifestation of LIPA deficiency, leading to the accumulation of cholesteryl esters and triglycerides in most tissues of the body. WOD occurs in infancy and is nearly always fatal before the age of 1 year.
    Defects in LIPA are the cause of cholesteryl ester storage disease (CESD) [MIM:278000]. CESD is a mild manifestation of LIPA deficiency, leading to the accumulation of cholesteryl esters and triglycerides in most tissues of the body. It is characterized by late-onset.
  • 序列相似性
    Belongs to the AB hydrolase superfamily. Lipase family.
  • 细胞定位
    Lysosome.
  • Information by UniProt

图片

  • 12.5% SDS-PAGE analysis of ab152501 stained with Coomassie Blue.

文献

ab152501 has not yet been referenced specifically in any publications.

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