Anti-Acid sphingomyelinase antibody [mAbcam74281] (ab74281)

Overview

• Product nameAnti-Acid sphingomyelinase antibody [mAbcam74281]See all Acid sphingomyelinase primary antibodies ...
• Description
  Mouse monoclonal [mAbcam74281] to Acid sphingomyelinase
• Tested applicationsWB, Flow Cyt more details
• Species reactivity
  Reacts with: Human
  
• Immunogen

  Synthetic peptide conjugated to KLH derived from within residues 1 - 100 of Human Acid sphingomyelinase.

  Read Abcam's proprietary immunogen policy

• Positive controlThis antibody gave a positive signal in the following whole cell lysates: HepG2; A431; MCF7; HeLa; THP1.
• General notesWe can conjugate this antibody to FITC for you (please see ab150251 for details).

Properties

• FormLiquid
• Storage instructionsStore at +4°C short term (1-2 weeks). Aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
• Storage bufferPreservative: 0.02% Sodium Azide
  Constituents: PBS, pH 7.4
• Concentration information loading...
• PurityIgG fraction
• Clonality Monoclonal
• Clone numbermAbcam74281
• MyelomaSp2/0-Ag14
• IsotypeIgG2a
• Light chain typekappa
• Research Areas
  • Metabolism
  • Types of disease
  • Cancer

  • Signal Transduction
  • Metabolism
  • Lipid metabolism

  • Neuroscience
  • Neurology process
  • Neurodegenerative disease
  • Other

  • Cell Biology
  • Apoptosis
  • Intracellular
  • Associated Proteins

Applications

Target

• 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
• Database links
  • Entrez Gene: 6609 Human
  • Omim: 607608 Human
  • SwissProt: P17405 Human
  • Unigene: 498173 Human

• Alternative names
  Acid sphingomyelinase antibodyASM antibodyASM_HUMAN antibody

  aSMase antibodyNPD antibodySmpd1 antibodySphingomyelin phosphodiesterase 1 acid lysosomal antibodySphingomyelin phosphodiesterase antibody

  see all

Anti-Acid sphingomyelinase antibody [mAbcam74281] images

• 

  Western blot - Acid sphingomyelinase antibody [mAbcam74281] (ab74281)
  All lanes : Anti-Acid sphingomyelinase antibody [mAbcam74281] (ab74281) at 5 µg/ml
  
  Lane 1 : HepG2 (Human hepatocellular liver carcinoma cell line) Whole Cell Lysate
  Lane 2 : A431 (Human epithelial carcinoma cell line) Whole Cell Lysate
  Lane 3 : MCF7 (Human breast adenocarcinoma cell line) Whole Cell Lysate
  
  Lysates/proteins at 10 µg per lane.
  
  Secondary
  Goat polyclonal Secondary Antibody to Mouse IgG - H&L (HRP), pre-adsorbed (ab97040) at 1/5000 dilution
  developed using the ECL technique
  
  Performed under reducing conditions.
  
  Predicted band size : 70 kDa
  Observed band size : 65 kDa (why is the actual band size different from the predicted?)
  
  
  Exposure time : 1 minute
• 

  Flow Cytometry-Anti-Acid sphingomyelinase antibody [mAbcam74281](ab74281)
  Overlay histogram showing HeLa cells stained with ab74281 (red line). The cells were fixed with 80% methanol (5 min) and then permeabilized with 0.1% PBS-Tween for 20 min. The cells were then incubated in 1x PBS / 10% normal goat serum / 0.3M glycine to block non-specific protein-protein interactions followed by the antibody (ab74281, 1µg/1x10⁶ cells) for 30 min at 22ºC. The secondary antibody used was DyLight® 488 goat anti-mouse IgG (H+L) (ab96879) at 1/500 dilution for 30 min at 22ºC. Isotype control antibody (black line) was mouse IgG2a [ICIGG2A] (ab91361, 1µg/1x10⁶ cells) used under the same conditions. Acquisition of >5,000 events was performed.