Recombinant Human SMPD1/ASM Protein (His Tag)

SKU: PKSH030434

Size: 50μg

Storage: Store at < -20°C, stable for 6 months. Please minimize freeze-thaw cycles.

Shipping: This product is provided as liquid. It is shipped at frozen temperature with blue ice/gel packs. Upon receipt, store it immediately at < - 20°C.

Exp date: 12 months

Category ID_II: Recombinant Proteins

Category ID_III: Others

Abbreviation:

Target Synonym: ASM;ASMASE;NPD

Research Areas: Cell Biology,Neuroscience,Signal Transduction,Metabolism

Conjugation:

Target Species: Human

Expression Host: Baculovirus-Insect Cells

Application: Enzyme

Fusion tag: C-His

UNIProt ID: P17405

Accession: NP_000534.3

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.

Concentration:

Activity: Measured by its ability to cleave 2-N-Hexadecanoylamino-4-nitrophenylphosphorylcholine (HNPPC). The specific activity is > 1000 pmol/min/μg.

Sequence: Met 1-Pro628

Purity: > 90 % as determined by reducing SDS-PAGE.

Formulation: Supplied as sterile 20 mM Tris, 500 mM NaCl, 25 % glycerol, pH 7.5.

Reconstitution: Not Applicable

Endotoxin: < 1.0 EU per μg of the protein as determined by the LAL method.

Calculated MW: 66.3 kDa

ObservedMW: