Recombinant Human SMPD1/ASM Protein (His Tag)

Specifications

Catalog number

PKSH030434

Size / count

50 µg

Dry ice

No

Hazardous

No

Discontinued

No

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.

Synonyms

ASM; ASMASE; NPD

UniProt

P17405

Accession Number

NP_000534.3

Expression System

Baculovirus-Insect Cells

Tag

C-His

Sequence

Met 1-Pro628

Applications

Enzyme

Field of Research

Cell Biology, Neuroscience, Signal Transduction, Metabolism

Endotoxin

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

Purity

> 90 % as determined by reducing SDS-PAGE.

Bioactivity

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

Reconstitution

Not Applicable

Shipping Conditions

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.

Storage Conditions

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

Calculated Molecular Weight

66.3 kDa

Formulation

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

Species

Human