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：Mouse
発現宿主：Baculovirus-Insect Cells
純度：＞85 ％ as determined by reducing SDS-PAGE.
エンドトキシン濃度：＜1.0 EU per μg of the protein as determined by the LAL method.
アクセッションNo.：Q04519
アミノ酸配列：Met 1-Leu 626
遺伝子構造：A DNA sequence encoding the mouse SMPD1 （Q04519） （Met 1-Leu 626） was expressed,with a C-terminal polyhistidine tag.
保存条件：Generally, lyophilized proteins are stable for up to 12 months when stored at -20 to -80℃. Reconstituted protein solution can be stored at 4-8℃ for 2-7 days. Aliquots of reconstituted samples are stable at ＜ -20℃ for 3 months.
