Anti-Acid sphingomyelinase antibody [mAbcam74281] (ab74281)
- Product nameAnti-Acid sphingomyelinase antibody [mAbcam74281]See all Acid sphingomyelinase primary antibodies ...
- DescriptionMouse monoclonal [mAbcam74281] to Acid sphingomyelinase
- Tested applicationsWB, Flow Cyt more details
- Species reactivityReacts with: Human
Synthetic peptide conjugated to KLH derived from within residues 1 - 100 of Human Acid sphingomyelinase.
- 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).
- 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
- Light chain typekappa
- Research Areas
Our Abpromise guarantee covers the use of ab74281 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||WB: Use a concentration of 5 µg/ml. Detects a band of approximately 65 kDa (predicted molecular weight: 70 kDa).|
|Flow Cyt||Flow Cyt: Use 1µg for 106 cells.|
- 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.
- Acid sphingomyelinase antibodyASM antibodyASM_HUMAN antibody
- aSMase antibodyNPD antibodySmpd1 antibodySphingomyelin phosphodiesterase 1 acid lysosomal antibodySphingomyelin phosphodiesterase antibody
Anti-Acid sphingomyelinase antibody [mAbcam74281] images
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.
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
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.
References for Anti-Acid sphingomyelinase antibody [mAbcam74281] (ab74281)
ab74281 has not yet been referenced specifically in any publications.