Acid sphingomyelinase/SMPD1 Protein, Mouse, Recombinant (His)

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.

Pack Size	Availability	Price/USD
50 μg	In stock	$ 386.00
100 μg	5 days	$ 660.00
200 μg	5 days	$ 1,120.00
500 μg	5 days	$ 2,270.00

Biological Information

Measured by its ability to cleave 2-N-Hexadecanoylamino-4-nitrophenylphosphorylcholine(HNPPC). The specific activity is > 1,000 pmoles/min/μg.

Description

Species	Mouse
Expression System	Baculovirus-Insect Cells
Tag	His
Accession Number	Q04519
Synonyms	aSMase, ASM, Zn-SMase, A-SMase, sphingomyelin phosphodiesterase 1
Construction	A DNA sequence encoding the mouse SMPD1 (Q04519) (Met 1-Leu 626) was expressed，with a C-terminal polyhistidine tag.
Protein Purity	> 85 % as determined by SDS-PAGE
Molecular Weight	Approxiamtely 66.3 kDa

Endotoxin	< 1.0 EU per μg of the protein as determined by the LAL method.
Formulation	Supplied as sterile 20mM Tris, 500mM NaCl, 10% glycerol, pH 8.0, 0. 1% Tween20Please contact us for any concerns or special requirements. Please refer to the specific buffer information in the hard copy of CoA.
Reconstitution	A hardcopy of COA with reconstitution instruction is sent along with the products. Please refer to it for detailed information.
Stability & Storage	Samples are stable for up to twelve months from date of receipt at -20℃ to -80℃. Store it under sterile conditions at -20℃ to -80℃. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.
Shipping	Solution. It is shipped out with blue ice.
Research 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.