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- サプライヤー
- TargteMol
- カタログNo.
- TMPY-02585
- 製品名称
- Acid sphingomyelinase/SMPD1 Protein, Mouse, Recombinant (His)
- タンパク質名
- Acid sphingomyelinase/SMPD1
- Species
- Mouse
- HOST
- Baculovirus Insect Cells
- 50μg: -
- お問い合わせ
Overview
| Synonyms | aSMase, ASM, Zn-SMase, A-SMase, sphingomyelin phosphodiesterase 1 |
|---|---|
| Characteristics | Measured by its ability to cleave 2-N-Hexadecanoylamino-4-nitrophenylphosphorylcholine(HNPPC). The specific activity is > 1,000 pmoles/min/μg. |
| Endotoxin Level | < 1.0 EU/μg of the protein as determined by the LAL method. |
| Purity | 90.70% |
| Description | 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 | 5175 |
| URL | https://www.targetmol.com/recombinant-protein/acid_sphingomyelinase_smpd1_protein_mouse_recombinant_his_tag_ |
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