Human SMPD1 Protein (His Tag)
ASM,ASMASE,NPD
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| Catalog Number | P11087-H08B1 |
|---|---|
| Organism Species | Human |
| Host | Baculovirus-Insect Cells |
| Synonyms | ASM,ASMASE,NPD |
| Molecular Weight | The recombinant human SMPD1 consists of 593 amino acids and predicts a molecular mass of 66.3 kDa. |
| predicted N | Leu 47 |
| SDS-PAGE |  |
| Purity | > 95 % as determined by SDS-PAGE. |
| Protein Construction | A DNA sequence encoding the human SMPD1 (BAD93012.1) (Met1-Pro628) was expressed with a polyhistidine tag at the C-terminus. |
| Bio-activity | |
| Research Area | Cancer |Signal transduction |Metabolism |Lipid metabolism |
| Formulation | Lyophilized from sterile 20 mM Tris, 500 mM NaCl, 25 % glycerol, pH 7.5. 1. Normally 5 % - 8 % trehalose and mannitol are added as protectants before lyophilization. Specific concentrations are included in the hardcopy of COA. |
| 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. |
| Reference |