Overview

  • Product name

    Anti-Lysosomal acid lipase/LAL antibody [1F9]
    See all Lysosomal acid lipase/LAL primary antibodies
  • Description

    Mouse monoclonal [1F9] to Lysosomal acid lipase/LAL
  • Host species

    Mouse
  • Tested applications

    Suitable for: WB, IHC-P, IPmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Cynomolgus monkey
  • Immunogen

    Recombinant full length protein (GST-tag) corresponding to Human Lysosomal acid lipase/LAL aa 1-399. (AAH12287).
    Sequence:

    MKMRFLGLVV CLVLWTLHSE GSGGKLTAVD PETNMNVSEI ISYWGFPSEE YLVETEDGYI LCLNRIPHGR KNHSDKGPKP VVFLQHGLLA DSSNWVTNLA NSSLGFILAD AGFDVWMGNS RGNTWSRKHK TLSVSQDEFW AFSYDEMAKY DLPASINFIL NKTGQEQVYY VGHSQGTTIG FIAFSQIPEL AKRIKMFFAL GPVASVAFCT SPMAKLGRLP DHLIKDLFGD KEFLPQSAFL KWLGTHVCTH VILKELCGNL CFLLCGFNER NLNMSRVDVY TTHSPAGTSV QNMLHWSQAV KFQKFQAFDW GSSAKNYFHY NQSYPPTYNV KDMLVPTAVW SGGHDWLADV YDVNILLTQI TNLVFHESIP EWEHLDFIWG LDAPWRLYNK IINLMRKYQ


    Database link: P38571

  • Positive control

    • Human spleen tissue; Lysosomal acid lipase/LAL transfected 293T lysate.
  • General notes

     This product was previously labelled as Lysosomal acid lipase

     

Properties

Applications

Our Abpromise guarantee covers the use of ab219113 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
WB Use at an assay dependent concentration. Predicted molecular weight: 45 kDa.
IHC-P Use a concentration of 10 µg/ml.
IP Use at an assay dependent concentration.

Target

  • 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
  • Database links

    • Alternative names

      • Acid cholesteryl ester hydrolase antibody
      • CESD antibody
      • cholesterol ester hydrolase antibody
      • cholesterol ester storage disease antibody
      • Cholesteryl esterase antibody
      • Hydrolase deficiency antibody
      • LAL antibody
      • LAL deficiency cholesterol ester antibody
      • LICH_HUMAN antibody
      • lipA antibody
      • LIPA deficiency antibody
      • Lipase A antibody
      • lipase A, lysosomal acid, cholesterol esterase antibody
      • lysosomal acid lipase antibody
      • lysosomal acid lipase deficiency antibody
      • Lysosomal acid lipase/cholesteryl ester hydrolase antibody
      • Sterol esterase antibody
      see all

    Images

    • All lanes : Anti-Lysosomal acid lipase/LAL antibody [1F9] (ab219113)

      Lane 1 : Lysosomal acid lipase/LAL transfected 293T lysate
      Lane 2 : Non-transfected 293T lysate

      Predicted band size: 45 kDa

    • Immunohistochemical analysis of formalin-fixed, paraffin-embedded Human spleen tissue labeling Lysosomal acid lipase/LAL with ab219113 at 10µg/ml.

    • Immunoprecipitation analysis of Lysosomal acid lipase/LAL transfected lysate using ab219113 and Protein A Magnetic Bead, and immunoblotted with Lysosomal acid lipase/LAL rabbit polyclonal antibody

    References

    ab219113 has not yet been referenced specifically in any publications.

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