bs-6318R [Primary Antibody]
Acid sphingomyelinase Antibody
www.biossusa.com
[email protected]
800.501.7654 [DOMESTIC]
+1.781.569.5821 [INTERNATIONAL]
DATASHEET

Host: Rabbit

Target Protein: Acid sphingomyelinase

Immunogen Range: 201-300/629


Clonality: Polyclonal

Isotype: IgG

Entrez Gene: 6609

Swiss Prot: P17405

Source: KLH conjugated synthetic peptide derived from human Acid sphingomyelinase

Purification: Purified by Protein A.

Storage Buffer: 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Storage: Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

Background:

Converts sphingomyelin to ceramide. Also has phospholipase C activities toward 1,2-diacylglycerolphosphocholine and 1,2-diacylglycerolphosphoglycerol. Isoform 2 and isoform 3 have lost catalytic activity.Involvement in disease: Defects in SMPD1 are the cause of Niemann-Pick disease type A (NPDA) ; also known as Niemann-Pick disease classical infantile form. It is an early-onset lysosomal storage disorder caused by failure to hydrolyze sphingomyelin to ceramide. It results in the accumulation of sphingomyelin and other metabolically related lipids in reticuloendothelial and other cell types throughout the body, leading to cell death. Niemann-Pick disease type A is a primarily neurodegenerative disorder characterized by onset within the first year of life, mental retardation, digestive disorders, failure to thrive, major hepatosplenomegaly, and severe neurologic symptoms. The severe neurological disorders and pulmonary infections lead to an early death, often around the age of four. Clinical features are variable. A phenotypic continuum exists between type A (basic neurovisceral) and type B (purely visceral) forms of Niemann-Pick disease, and the intermediate types encompass a cluster of variants combining clinical features of both types A and B.

Size: 100ul

Concentration: 1ug/ul

Applications: WB(1:300-5000)
ELISA(1:500-1000)
IHC-P(1:200-400)
IHC-F(1:100-500)
IF(IHC-P)(1:50-200)
IF(IHC-F)(1:50-200)
IF(ICC)(1:50-200)
ICC(1:100-500)

Predicted Molecular Weight: 64


Cross Reactive Species: Human
Mouse
Rat

Predicted Cross Reactive Species: Dog
Cow
Pig
Rabbit

For research use only. Not intended for diagnostic or therapeutic use.

PRODUCT SPECIFIC PUBLICATIONS
  • Pe?ate T et al. Lipid-Iron Nanoparticle with a Cell Stress Release Mechanism Combined with a Local Alternating Magnetic Field Enables Site-Activated Drug ReleaseCancers (Basel).2020 Dec 14;12(12):3767.Read more>>
  • Anastasia M. Ravodina. et al. Facile Cholesterol Loading with a New Probe ezFlux Allows for Streamlined Cholesterol Efflux Assays. Acs Omega. 2020;5(36):23289C23298Read more>>
  • Tuula Penate Medina. et al. Utilizing Sphingomyelinase Sensitizing Liposomes in Imaging Intestinal Inflammation in Dextran Sulfate Sodium-Induced Murine Colitis. Biomedicines. 2022 Feb;10(2):413Read more>>
VALIDATION IMAGES

Lane 1: A431 lysates probed with Acid sphingomyelinase Antibody (bs-6318R) at 1:300 overnight at 4˚C. Followed by a conjugated secondary antibody at 1:5000 for 90 min at 37˚C.