Product nameHuman Acid sphingomyelinase Antibody Pair - BSA and Azide free (SMPD1)
See all Acid sphingomyelinase kits
Assay typeELISA set
Range125 pg/ml - 8000 pg/ml
Species reactivityReacts with: Human
Both capture and detector antibodies are recombinant rabbit monoclonal antibodies delivering consistent, specific, and sensitive results.
The Antibody Pair can be used to quantify Human Acid sphingomyelinase. BSA and Azide free antibody pairs include unconjugated capture and detector antibodies suitable for sandwich ELISAs. The antibodies are provided at an approximate concentration of 1 mg/ml as measured by the protein A280 method. The recommended antibody orientation is based on internal optimization for ELISA-based assays. Antibody orientation is assay dependent and needs to be optimized for each assay type.
For additional information on the performance of the antibody pair, see the equivalent SimpleStep ELISA® Kit (ab277075), which uses the same antibodies. However, due to differences in their formulation, this antibody pair cannot be used with the consumables provided with our SimpleStep ELISA Kits. Please note that the range provided for the pairs is only an estimation based on the performance of the related product using the same antibody pair. Performance of the antibody pair will depend on the specific characteristics of your assay. We guarantee the product works in sandwich ELISA, but we do not guarantee the sensitivity or dynamic range of the antibody pair in your assay.
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Tested applicationsSuitable for: Sandwich ELISAmore details
Storage instructionsStore at +4°C. Please refer to protocols.
Components 10 x 96 tests Human Acid sphingomyelinase Capture Antibody (unconjugated) 1 x 100µg Human Acid sphingomyelinase Detector Antibody (unconjugated) 1 x 100µg
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
Our Abpromise guarantee covers the use of ab253462 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|Sandwich ELISA||Use at an assay dependent concentration.|
To learn more about the advantages of recombinant antibodies see here.
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
ab253462 has not yet been referenced specifically in any publications.