Key features and details
- Detection method: Colorimetric
Product nameAlkaline Sphingomyelinase Assay Kit
See all Acid sphingomyelinase kits
Alkaline Sphingomyelinase (AlkSMase) Assay Kit (ab241039) provides a simple, high throughput adaptable means to identify and quantify alkaline sphingomyelinase activity in a variety of samples without influence from neutral or acid sphingomyelinase. Upon incubation with substrate, the hydrolysis produces an intermediate that reacts with the probe, generating a colorimetric signal.
The assay can detect as low as 0.5 µU of alkaline sphingomyelinase activity.
Storage instructionsStore at -20°C. Please refer to protocols.
Components Identifier 100 tests AlkSMase Assay Buffer WM 1 x 25ml AlkSMase Developer Buffer NM 1 x 5ml AlkSMase Enzyme Mix I (Lyophilized) Green 1 vial AlkSMase Enzyme Mix II (Lyophilized) White 1 vial AlkSMase Positive Control (Lyophilized) Orange 1 vial AlkSMase Probe (in DMSO) Red 1 x 200µl AlkSMase Substrate (Lyophilized) Blue 1 vial Choline Standard (Lyophilized) Yellow 1 vial
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.
- Information by UniProt
- Acid sphingomyelinase
Choline standard curve.
Alkaline Sphingomyelinase activity of NPP7. Hydrolysis was allowed to proceed for 1 hr. Neutral Sphingomyelinase (nSMase, purple bar) showed minimal activity after the same incubation time.
Activity determination of intestinal tissue lysate. For this experiment, 100 mg rat intestine was used, following Alkaline Sphingomyelinase Activity Assay Kit protocol with 2X protease inhibitor. Lysate was assayed and specific activity was determined to be 0.016 nmol/min/µg lysate.
ab241039 has not yet been referenced specifically in any publications.