Description
Recombinant Human SMPD1/ASM Protein (His Tag) | PKSH030434 | Gentaur US, UK & Europe Disrtribition
Synonyms: ASM;ASMASE;NPD
Active Protein: N/A
Activity: A DNA sequence encoding the human SMPD1 (BAD93012.1) (Met1-Pro628) was expressed with a polyhistidine tag at the C-terminus.
Protein Construction: A DNA sequence encoding the human SMPD1 (BAD93012.1) (Met1-Pro628) was expressed with a polyhistidine tag at the C-terminus.
Fusion Tag: C-His
Species: Human
Expressed Host: Baculovirus-Insect Cells
Shipping: This product is provided as lyophilized powder which is shipped with ice packs.
Purity: > 95 % as determined by reducing SDS-PAGE.
Endotoxin: < 1.0 EU per µg as determined by the LAL method.
Stability and Storage: 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.
Molecular Mass: 66.3 kDa
Formulation: Lyophilized from sterile 20 mM Tris, 500 mM NaCl, 25 % glycerol, pH 7.5
Reconstitution: Please refer to the printed manual for detailed information.
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.
Research Area: N/A
