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Anti-Acid sphingomyelinase/SMPD1 Antibody (9W945) is a Mouse antibody targeting Acid sphingomyelinase/SMPD1. Anti-Acid sphingomyelinase/SMPD1 Antibody (9W945) can be used in ELISA.
Description | Anti-Acid sphingomyelinase/SMPD1 Antibody (9W945) is a Mouse antibody targeting Acid sphingomyelinase/SMPD1. Anti-Acid sphingomyelinase/SMPD1 Antibody (9W945) can be used in ELISA. |
Ig Type | Monoclonal Mouse IgG1 |
Clone | 9W945 |
Reactivity | Human |
Application | ELISA |
Recommended Dose | ELISA: 1:1000-1:2000 |
Antibody Type | Monoclonal |
Host Species | Mouse |
Construction | This antibody was produced from a hybridoma resulting from the fusion of a mouse myeloma with B cells obtained from a mouse immunized with purified, recombinant Human SMPD1 (rh SMPD1; TMPY-01159; NP_000534.3; Met1-Pro628). The IgG fraction of the cell culture supernatant was purified by Protein A affinity chromatography. |
Purification | Protein A |
Appearance | Liquid |
Formulation | 0.2 μm filtered solution in PBS |
Research Background | Sphingomyelin phosphodiesterase 1 (SMPD1) , also known as ASM ( acid sphingomyelinase ), is a member of the acid sphingomyelinase family of enzymes. Three isoforms have been identified, isoform 1 is 631 amino acids (aa) in length as the pro form, while Isoform 2 and isoform 3 have lost catalytic activity. The active SMPD1 isoform 1 contains one saposin B-type domain that likely interacts with sphingomyelin, and a catalytic region. Human SMPD1 is 86% aa identical to mouse SMPD1. SMPD1 is a monomeric lysosomal enzyme that converts sphingomyelin (a plasma membrane lipid ) into ceramide through the removal of phosphorylcholine. This generates second messenger components that participate in signal transduction. Defects in SMPD1 are the cause of Niemann-Pick disease type A (NPA) and type B (NPB), also known as Niemann-Pick disease classical infantile form and Niemann-Pick disease visceral form. Niemann-Pick disease is a clinically and genetically heterogeneous recessive disorder. NPB has little if any neurologic involvement and patients may survive into adulthood. |
Conjucates | Unconjugated |
Immunogen | Recombinant Human SMPD1 Protein (TMPY-01159) |
Antigen Species | Human |
Stability & Storage | Store at 2°C-8°C for 1 month. Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles. Preservative-Free. |
Transport | Shipping with blue ice. |
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