Acid sphingomyelinase Antibody, Biotin Conjugated
Applications
Reactivity
Predicted Reactivity
| Overview | |
| Catalog # | bs-6318R-Biotin |
| Product Name | Acid sphingomyelinase Antibody, Biotin Conjugated |
| Applications |
WB
ELISA
IHC-P
IHC-F
|
| Reactivity | Human, Mouse, Rat |
| Predicted Reactivity | Dog, Cow, Pig, Rabbit |
| Specifications | |
| Conjugation | Biotin |
| Host | Rabbit |
| Source | KLH conjugated synthetic peptide derived from human Acid sphingomyelinase |
| Clonality | Polyclonal |
| Isotype | IgG |
| Concentration | 1ug/ul |
| Purification | Purified by Protein A. |
| Storage Buffer | Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Storage Condition | Store at -20C for 12 months. |
| 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 |
Frequently asked questions
Which applications is Acid sphingomyelinase Antibody, Biotin Conjugated (bs-6318R-Biotin) validated for, and at what dilution?
bs-6318R-Biotin is validated for WB (1:300-5000); ELISA (1:500-1000); IHC-P (1:200-400); IHC-F (1:100-500). These are recommended starting dilutions; optimal dilutions should be determined by the end user.
Which species does Acid sphingomyelinase Antibody, Biotin Conjugated (bs-6318R-Biotin) 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-Biotin be stored?
Store at -20C for 12 months. Storage buffer: Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
What immunogen was used to raise Acid sphingomyelinase Antibody, Biotin Conjugated (bs-6318R-Biotin)?
KLH conjugated synthetic peptide derived from human Acid sphingomyelinase