Recombinant Human Lysosomal acid lipase protein (ab152501)

Overview

  • Product name
    Recombinant Human Lysosomal acid lipase protein
  • Protein length
    Full length protein

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Accession
    • Species
      Human
    • Sequence
      MKMRFLGLVVCLVLWPLHSEGSGGKLTALDPETNMNVSEIISYWGFPSEE YLVETEDGYILCLNRIPHGRKNHSDKGPKPVVFLQHGLLADSSNWVTNLA NSSLGFILADAGFDVWMGNSRGNTWSRKHKTLSVSQDEFWAFSYDEMAKY DLPASINFILNKTGQEQVYYVGHSQGTTIGFIAFSQIPELAKRIKMFFAL GPVASVAFCTSPMAKLGRLPDHLIKDLFGDKEFLPQSAFLKWLGTHVCTH VILKELCGNLCFLLCGFNERNLNMSRVDVYTTHSPAGTSVQNMLHWSQAV KFQKFQAFDWGSSAKNYFHYNQSYPPTYNVKDMLVPTAVWSGGHDWLADV YDVNILLTQITNLVFHESIPEWEHLDFIWGLDAPWRLYNKIINLMRKYQ
    • 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
    Protein concentration is above or equal to 0.05 µg/µl.
  • 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

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

References

ab152501 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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