Overview

  • Product name

    Anti-Acid sphingomyelinase antibody
    See all Acid sphingomyelinase primary antibodies
  • Description

    Rabbit polyclonal to Acid sphingomyelinase
  • Host species

    Rabbit
  • Tested applications

    Suitable for: IHC-P, WBmore details
  • Species reactivity

    Reacts with: Human
  • Immunogen

    Recombinant fragment within Human Acid sphingomyelinase (internal sequence). The exact sequence is proprietary.
    Database link: P17405

  • Positive control

    • WB: Raji whole cell lysate. IHC-P: Human breast cancer tissue.

Properties

Applications

Our Abpromise guarantee covers the use of ab227966 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
IHC-P 1/100 - 1/1000.
WB 1/500 - 1/3000. Predicted molecular weight: 70 kDa.

Target

  • Function

    Converts sphingomyelin to ceramide. Also has phospholipase C activities toward 1,2-diacylglycerolphosphocholine and 1,2-diacylglycerolphosphoglycerol. Isoform 2 and isoform 3 have lost catalytic activity.
  • Involvement in disease

    Defects in SMPD1 are the cause of Niemann-Pick disease type A (NPDA) [MIM:257200]; also known as Niemann-Pick disease classical infantile form. It is an early-onset lysosomal storage disorder caused by failure to hydrolyze sphingomyelin to ceramide. It results in the accumulation of sphingomyelin and other metabolically related lipids in reticuloendothelial and other cell types throughout the body, leading to cell death. Niemann-Pick disease type A is a primarily neurodegenerative disorder characterized by onset within the first year of life, mental retardation, digestive disorders, failure to thrive, major hepatosplenomegaly, and severe neurologic symptoms. The severe neurological disorders and pulmonary infections lead to an early death, often around the age of four. Clinical features are variable. A phenotypic continuum exists between type A (basic neurovisceral) and type B (purely visceral) forms of Niemann-Pick disease, and the intermediate types encompass a cluster of variants combining clinical features of both types A and B.
    Defects in SMPD1 are the cause of Niemann-Pick disease type B (NPDB) [MIM:607616]; also known as Niemann-Pick disease visceral form. It is a late-onset lysosomal storage disorder caused by failure to hydrolyze sphingomyelin to ceramide. It results in the accumulation of sphingomyelin and other metabolically related lipids in reticuloendothelial and other cell types throughout the body, leading to cell death. Clinical signs involve only visceral organs. The most constant sign is hepatosplenomegaly which can be associated with pulmonary symptoms. Patients remain free of neurologic manifestations. However, a phenotypic continuum exists between type A (basic neurovisceral) and type B (purely visceral) forms of Niemann-Pick disease, and the intermediate types encompass a cluster of variants combining clinical features of both types A and B. In Niemann-Pick disease type B, onset of the first symptoms occurs in early childhood and patients can survive into adulthood.
  • Sequence similarities

    Belongs to the acid sphingomyelinase family.
    Contains 1 saposin B-type domain.
  • Cellular localization

    Lysosome.
  • Information by UniProt
  • Database links

  • Alternative names

    • Acid sphingomyelinase antibody
    • ASM antibody
    • ASM_HUMAN antibody
    • aSMase antibody
    • NPD antibody
    • Smpd1 antibody
    • Sphingomyelin phosphodiesterase 1 acid lysosomal antibody
    • Sphingomyelin phosphodiesterase antibody
    see all

Images

  • Anti-Acid sphingomyelinase antibody (ab227966) at 1/1000 dilution + Raji (human Burkitt's lymphoma cell line) whole cell lysate at 30 µg

    Secondary
    HRP-conjugated anti-rabbit IgG

    Predicted band size: 70 kDa



    10% SDS PAGE gel.

  • Paraffin-embedded human breast cancer tissue stained for Acid sphingomyelinase using ab227966 at 1/500 dilution in immunohistochemical analysis.

References

ab227966 has not yet been referenced specifically in any publications.

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