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Acid sphingomyelinase protein (Tagged) (ab132844)

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Overview

  • Product nameAcid sphingomyelinase protein (Tagged)See all Acid sphingomyelinase proteins and peptides ...
  • Protein descriptionRecombinant full length protein of Human Acid sphingomyelinase with proprietary N terminal tag; Predicted MW 65.78 kDa (inclusive of tag).
  • Molecular weight65.780kDa inclusive of tags
  • Protein length364 amino acids
  • Expression hostWheat germ

    • Properties

  • FormLiquid
  • Storage instructionsShipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
  • Storage bufferpH: 8.00
    Constituents: 0.31% Glutathione, 0.79% Tris HCl
    Note: Reduced glutathione
  • Concentration information loading...
  • Sequence notesMPRYGASLRQSCPRSGREQGQDGTAGAPGLLWMGLALALA LALALALSDSRVLWAPAEAHPLSPQGHPARLHRIVPRLRD VFGWGNLTCPICKGLFTAINLGLKKEPNVARVGSVAIKLC NLLKIAPPAVCRSIVHLFEDDMVEVWRRSVLSPSEACGLL LGSTCGHWDIFSSWNISLPTVPKPPPKPPSPPAPGAPVSR ILFLTDLHWDHDYLEGTDPDCADPLCCRRGSGLPPASRPG AGYWGEYSKCDLPLRTLESLLSGLGPAGPFDMVYWTGDIP AHDVWHQTRQDQLRALTTVTALVRKFLGPVPVYPAVGNHE STPVNSFPPPFIEGNHSSRWLYEAMAKAWEPWLPAEALRT LRCI
  • Research Areas

    • Applications

    Our Abpromise guarantee covers the use of ab132844 in the following tested applications.

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

    Application Notes
    WB WB: Use at an assay dependent concentration.
    SDS-PAGE SDS-PAGE: Use at an assay dependent concentration.
    ELISA ELISA: Use at an assay dependent concentration.
  • Application notesThis peptide can be used with studies using ab170579.

    • Protein info

    • Alternative names
        Acid sphingomyelinaseASMASM_HUMAN
        aSMaseNPDSmpd1Sphingomyelin phosphodiesteraseSphingomyelin phosphodiesterase 1 acid lysosomal
      see all
  • FunctionConverts 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 diseaseDefects 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 similaritiesBelongs to the acid sphingomyelinase family.
    Contains 1 saposin B-type domain.
  • Cellular localizationLysosome.

    • Target information above from: UniProt accession P17405 The UniProt Consortium
    The Universal Protein Resource (UniProt) in 2010
    Nucleic Acids Res. 38:D142-D148 (2010) .

    Information by UniProt

    Acid sphingomyelinase protein (Tagged) images

    • SDS-PAGE - Acid sphingomyelinase protein (Tagged) (ab132844)
      SDS-PAGE - Acid sphingomyelinase protein (Tagged) (ab132844)
      12.5% SDS-PAGE Stained with Coomassie Blue

    References for Acid sphingomyelinase protein (Tagged) (ab132844)

    ab132844 has not yet been referenced specifically in any publications.

    Publishing research using ab132844 ? Please let us know so that we can cite the reference in this datasheet

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