Overview

  • Product name
    Anti-Lysosomal acid lipase antibody
    See all Lysosomal acid lipase primary antibodies
  • Description
    Rabbit polyclonal to Lysosomal acid lipase
  • Host species
    Rabbit
  • Tested applications
    Suitable for: WBmore details
  • Species reactivity
    Reacts with: Mouse, Human
  • Immunogen

    Recombinant fragment corresponding to a region within amino acids 164-399 of Human Lysosomal acid lipase

  • Positive control
    • A549 whole cell lysate, H1299 whole cell lysate, mouse liver lysate

Properties

Applications

Our Abpromise guarantee covers the use of ab154356 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 1/500 - 1/3000. Predicted molecular weight: 45 kDa.

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

  • Anti-Lysosomal acid lipase antibody (ab154356) at 1/1000 dilution + Mouse liver whole cell lysate at 50 µg

    Predicted band size: 45 kDa



    10% SDS PAGE
  • All lanes : Anti-Lysosomal acid lipase antibody (ab154356) at 1/1000 dilution

    Lane 1 : A549 whole cell lysate
    Lane 2 : H1299 whole cell lysate

    Lysates/proteins at 30 µg per lane.

    Predicted band size: 45 kDa



    10% SDS PAGE

References

This product has been referenced in:
  • Llewellyn KJ  et al. A Fine Balance of Dietary Lipids Improves Pathology of a Murine Model of VCP-Associated Multisystem Proteinopathy. PLoS One 10:e0131995 (2015). WB ; Mouse . Read more (PubMed: 26134519) »

See 1 Publication for this product

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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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