Acid sphingomyelinase Antibody
Applications
Reactivity
Predicted Reactivity
| Overview | |
| Catalog # | bs-6318R-100uL |
| Product Name | Acid sphingomyelinase Antibody |
| Applications |
WB
ELISA
IHC-P
IHC-F
IF(IHC-P)
IF(IHC-F)
IF(ICC)
|
| Reactivity | Human, Mouse, Rat |
| Predicted Reactivity | Dog, Cow, Pig, Rabbit |
| Specifications | |
| Conjugation | Unconjugated |
| Host | Rabbit |
| Source | KLH conjugated synthetic peptide derived from human Acid sphingomyelinase |
| Immunogen Range | 201-300/629 |
| Clonality | Polyclonal |
| Isotype | IgG |
| Concentration | 1ug/ul |
| Purification | Purified by Protein A. |
| Storage Buffer | 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Storage Condition | Shipped at 4C. Store at -20C for one year. Avoid repeated freeze/thaw cycles. |
| Target | |
| Gene ID | 6609 |
| Swiss Prot | P17405 |
| Subcellular location | Cytoplasm |
| Synonyms | Acid sphingomyelinase; ASM; ASM_HUMAN; aSMase; NPD; Smpd1; Sphingomyelin phosphodiesterase 1 acid lysosomal; Sphingomyelin phosphodiesterase. |
| 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. |
| Application Dilution | |
| 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 |
Frequently asked questions
Which applications is Acid sphingomyelinase Antibody (bs-6318R) validated for, and at what dilution?
bs-6318R is validated for 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). These are recommended starting dilutions; optimal dilutions should be determined by the end user.
Which species does Acid sphingomyelinase Antibody (bs-6318R) react with?
Tested reactivity: Human, Mouse, Rat. Predicted reactivity (expected from sequence homology but not yet tested): Dog, Cow, Pig, Rabbit.
How should bs-6318R be stored?
Shipped at 4C. Store at -20C for one year. Avoid repeated freeze/thaw cycles. Storage buffer: 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
What immunogen was used to raise Acid sphingomyelinase Antibody (bs-6318R)?
KLH conjugated synthetic peptide derived from human Acid sphingomyelinase Immunogen range: 201-300/629.