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Rabbit anti Human Acid Sphingomyelinase


Catalogue number: X1695P

Synonyms
Acid Sphingomyelinase, aSMase, SMPD1, ASM, Sphingomyelin phosphodiesterase, ASM-1

IsotypeN/A
Product Type Polyclonal Antibody
Units100 µg
HostRabbit
Species reactivity Human
Application ELISA
Western Blot
Product Form
Unconjugated

Background
Human acid sphingomyelinase (sphingomyelin phosphodiesterase, ASM) is the lysosomal enzyme responsible for the hydrolysis of sphingomyelin to ceramide and phosphocholine. Converts sphingomyelin to ceramide. aSM also has phospholipase C activities toward 1,2-diacylglycerol-phosphocholine and 1,2-diacylglycerol-phosphoglycerol. The enzyme is a membrane-associated glycoprotein with a pH optimum of about 4.5 and a subunit molecular mass of about 72 kDa. In addition AtoS M, two other sphingomyelinases have been identified in man, a Mg2+- dependent neutral sphingomyelinase found primarily in brain and a Zn2+-dependent acid sphingomyelinase found primarily in serum. Although it is likely that the acid and neutral sphingomyelinases are coded by different genes, the molecular genetic relationship of these three biochemically distinct sphingomyelinases has not been determined. Understanding the role of these sphingomyelinases in the hydrolysis of sphingomyelin to ceramide will be an important step in the understanding of ceramide as it is further hydrolyzed to sphingosine, a neutral phospholipid which has been implicated in the regulation of protein kinase C-mediated signal transduction. Inherited deficiencies of ASM have been reported in man, deficient ASM activity results in the two major subtypes of Niemann-Pick disease (NPD).

Immunogen
Synthetic peptide derived from human acid sphingomyelinase protein.

Purification Method
Ammonium Sulfate Precipitation

Concentration
See vial for concentration

Formulation
Provided as solution in phosphate buffered saline with 0.08% sodium azide

Functional Analysis
Western Blotting

Applications
Antibody can be used for Western blots (5-10 µg/ml) and ELISA. Optimal concentration should be evaluated by serial dilutions.

Storage
Product should be stored at -20ºC. Aliquot to avoid freeze/thaw cycles

Ship Conditions
Ship at ambient temperature, freeze upon arrival

Product Stability
Products are stable for one year from purchase when stored properly

References
1. Human acid sphingomyelinase. Isolation, nucleotide sequence and expression of the full-length and alternatively spliced cDNAs.; Schuchman E.H., Suchi M., Takahashi T., Sandhoff K., Desnick R.J.; J. Biol. Chem. 266:8531-8539(1991). 2. Molecular cloning of the acid sphingomyelinase of the mouse and the organization and complete nucleotide sequence of the gene.; Newrzella D., Stoffel W.; Biol. Chem. Hoppe-Seyler 373:1233-1238(1992). 3. Cloning of a human acid sphingomyelinase cDNA with a new mutation that renders the enzyme inactive.; Ida H., Rennert O.M., Eto Y., Chan W.Y.; J. Biochem. 114:15-20(1993). 4. Isolation of cDNA clones encoding human acid sphingomyelinase: occurrence of alternatively processed transcripts.; Quintern L.E., Schuchman E.H., Levran O., Suchi M., Ferlinz K., Reinke H., Sandhoff K., Desnick R.J.; EMBO J. 8:2469-2473(1989). 5. Functional characterization of the N-glycosylation sites of human acid sphingomyelinase by site-directed mutagenesis.; Ferlinz K., Hurwitz R., Moczall H., Lansmann S., Schuchman E.H., Sandhoff K.; Eur. J. Biochem. 243:511-517(1997). 6. Human acid sphingomyelinase.; Lansmann S., Schuette C.G., Bartelsen O., Hoernschemeyer J., Linke T., Weisgerber J., Sandhoff K.;Eur. J. Biochem. 270:1076-1088(2003).

Caution
This product is intended FOR RESEARCH USE ONLY, and FOR TESTS IN VITRO, not for use in diagnostic or therapeutic procedures involving humans or animals.

Safety Datasheet(s) for this product:

Sodium Azide
/wp-content/uploads/2018/07/Antibody-SDS-with-Sodium-AzideV2.pdf

Rabbit anti Human Acid Sphingomyelinase

$355.00

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