Overview

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Accession
    • Species
      Human
    • Sequence
      PPWTEDCRKSTYPPSGPTYRGAVPWYTINLDLPPYKRWHELMLDKAPMLK VIVNSLKNMINTFVPSGKVMQVVDEKLPGLLGNFPGPFEEEMKGIAAVTD
    • Molecular weight
      37 kDa including tags
    • Amino acids
      25 to 124

Specifications

Our Abpromise guarantee covers the use of ab114948 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 mg/ml.
    Best used within three months from the date of receipt.
  • 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: 0.3% Glutathione, 0.79% Tris HCl

General Info

  • Alternative names
    • AC
    • ACDase
    • Acid CDase
    • Acid ceramidase
    • Acid ceramidase precursor
    • Acid ceramidase subunit beta
    • Acylsphingosine deacylase
    • ASAH
    • ASAH 1
    • ASAH1
    • ASAH1_HUMAN
    • FLJ21558
    • FLJ22079
    • N acylsphingosine amidohydrolase
    • N acylsphingosine amidohydrolase (acid ceramidase) 1
    • N acylsphingosine amidohydrolase 1
    • N-acylsphingosine amidohydrolase
    • N-acylsphingosine deacylase
    • PHP
    • PHP32
    • Putative 32 kDa heart protein
    • SMAPME
    see all
  • Function
    Hydrolyzes the sphingolipid ceramide into sphingosine and free fatty acid.
  • Tissue specificity
    Broadly expressed with highest expression in heart.
  • Involvement in disease
    Defects in ASAH1 are the cause of Farber lipogranulomatosis (FL) [MIM:228000]; also known as Farber disease (FD). This sphingolipid disease is characterized by subcutaneous lipid-loaded nodules, excruciating pain in the joints and extremities, marked accumulation of ceramide in lysosomes, and death by three years of age.
  • Sequence similarities
    Belongs to the acid ceramidase family.
  • Cellular localization
    Lysosome.
  • Information by UniProt

Images

  • 12.5% SDS-PAGE showing ab114948 at approximately 36.63kDa.
    Stained with Coomassie Blue.

References

ab114948 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

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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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