Anti-Acid sphingomyelinase antibody (bs-6318R)

Anti-Acid sphingomyelinase antibody

DATASHEET

Host:Rabbit

Target Protein:Acid sphingomyelinase

IR: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:201-300/629 

Purification:affinity purified by Protein A

Storage:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Shipped at 4℃. 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:1mg/ml

Applications:WB(1:500-2000)
ELISA(1:5000-10000)
IHC-P(1:100-500)
IHC-F(1:100-500)
Flow-Cyt(2ug/Test)
ICC(1:100-500)
IF(1:100-500)

Cross Reactive Species:Human
Mouse
Rat
Dog
Pig
Cow
Rabbit
.

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

VALIDATION IMAGES

Sample:
HepG2(human) cell Lysate at 30 ug
MCF-7(human) cell Lysate at 30 ug
A431(human) cell Lysate at 30 ug
Hale(human) cell Lysate at 30 ug
Primary: Anti- Acid sphingomyelinase (bs-6318R) at 1/300 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 64kD
Observed band size: 69 kD

Paraformaldehyde-fixed, paraffin embedded (Rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Acid sphingomyelinase) Polyclonal Antibody, Unconjugated (bs-6318R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.