Recombinant Human Lysosomal acid lipase/LAL protein (Tagged) (ab152501)

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SDS-PAGE - Recombinant Human Lysosomal acid lipase/LAL protein (ab152501)

    Key features and details

    • Expression system: Wheat germ
    • Tags: GST tag N-Terminus
    • Suitable for: ELISA, SDS-PAGE, WB

    Description

    • Product name

      Recombinant Human Lysosomal acid lipase/LAL protein (Tagged)

    • Expression system

      Wheat germ

    • Accession

      P38571

    • Protein length

      Full length protein

    • Animal free

      No

    • Nature

      Recombinant

      • Species

        Human

      • Sequence

        MKMRFLGLVVCLVLWPLHSEGSGGKLTALDPETNMNVSEIISYWGFPSEE YLVETEDGYILCLNRIPHGRKNHSDKGPKPVVFLQHGLLADSSNWVTNLA NSSLGFILADAGFDVWMGNSRGNTWSRKHKTLSVSQDEFWAFSYDEMAKY DLPASINFILNKTGQEQVYYVGHSQGTTIGFIAFSQIPELAKRIKMFFAL GPVASVAFCTSPMAKLGRLPDHLIKDLFGDKEFLPQSAFLKWLGTHVCTH VILKELCGNLCFLLCGFNERNLNMSRVDVYTTHSPAGTSVQNMLHWSQAV KFQKFQAFDWGSSAKNYFHYNQSYPPTYNVKDMLVPTAVWSGGHDWLADV YDVNILLTQITNLVFHESIPEWEHLDFIWGLDAPWRLYNKIINLMRKYQ

      • Predicted molecular weight

        70 kDa including tags

      • Amino acids

        1 to 399

      • Tags

        GST tag N-Terminus

    Associated products

    Specifications

    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.

    • Applications

      ELISA

      SDS-PAGE

      Western blot

    • Form

      Liquid

    • Additional notes

      This product was previously labelled as Lysosomal acid lipase.

       

    • Concentration information loading...

    Preparation and Storage

    • Stability and Storage

      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

    General Info

    • Alternative names

      • 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

    • Function

      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.

    • Involvement in disease

      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.

    • Sequence similarities

      Belongs to the AB hydrolase superfamily. Lipase family.

    • Cellular localization

      Lysosome.
    • Information by UniProt

    Images

    • SDS-PAGE - Recombinant Human Lysosomal acid lipase/LAL protein (ab152501)
      SDS-PAGE - Recombinant Human Lysosomal acid lipase/LAL protein (ab152501)
      12.5% SDS-PAGE analysis of ab152501 stained with Coomassie Blue.

    Protocols

    To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.

    Click here to view the general protocols

    References (0)

    Publishing research using ab152501? Please let us know so that we can cite the reference in this datasheet.

    ab152501 has not yet been referenced specifically in any publications.

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