特徴
- 背景: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.
仕様
- サイズ:50ug
- 種由来:Human
- 発現宿主:Baculovirus-Insect Cells
- 純度:> 95 % as determined by reducing SDS-PAGE.
- エンドトキシン濃度:< 1.0 EU per ug as determined by the LAL method.
- アクセッションNo.:BAD93012.1
- タグ種類:C-His
- アミノ酸配列:Met 1-Pro628
- 遺伝子構造:A DNA sequence encoding the human SMPD1 (BAD93012.1) (Met1-Pro628) was expressed with a polyhistidine tag at the C-terminus.
- 別名(遺伝子名):ASM;ASMASE;NPD
- 分子量:66.3 kDa
- 組成:Lyophilized from sterile 20 mM Tris, 500 mM NaCl, 25 % glycerol, pH 7.5
- 保存条件:Lyophilized proteins are stable for up to 12 months when stored at -20 to -80°C. Reconstituted protein solution can be stored at 4-8℃ for 2-7 days. Aliquots of reconstituted samples are stable at < -20℃ for 3 months.
- メーカーマニュアルリンク:https://www.elabscience.com/viewpdf-37231-Elabscience-PKSH030434.pdf
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商品のバリエーション (サイズ違い・スペック違い・オプション品など)
| 商品イメージ | アズワン品番 商品名 |
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89-7746-40
Recombinant Human SMPD1/ASM Protein (His Tag) 10ug
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89-7746-41
Recombinant Human SMPD1/ASM Protein (His Tag) 50ug
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