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Synonyms: NPD, ASMASE, ASM
| Pack Size | Price | USA Stock | Global Stock | Quantity |
|---|---|---|---|---|
| 50 μL | $221 | 7-10 days | 7-10 days | |
| 100 μL | $373 | 7-10 days | 7-10 days | |
| 200 μL | $527 | 7-10 days | 7-10 days |
| Description | Anti-Acid sphingomyelinase/SMPD1 Polyclonal Antibody is a Rabbit antibody targeting Acid sphingomyelinase/SMPD1. Anti-Acid sphingomyelinase/SMPD1 Polyclonal Antibody can be used in FCM, IF, IHC-Fr, IHC-P, WB. |
| Synonyms | NPD, ASMASE, ASM |
| Ig Type | IgG |
| Reactivity | Human,Rat (predicted:Mouse,Dog,Pig,Cow,Rabbit) |
| Application | |
| Recommended Dose | WB: 1:500-2000; IHC-P: 1:100-500; IHC-Fr: 1:100-500; IF: 1:100-500; FCM: 2ug/Test |
| Antibody Type | Polyclonal |
| Host Species | Rabbit |
| Subcellular Localization | Lysosome. |
| Construction | Polyclonal Antibody |
| Purification | Protein A purified |
| Appearance | Liquid |
| Formulation | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Concentration | 1 mg/mL |
| Research 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. |
| Immunogen | KLH conjugated synthetic peptide: human Acid sphingomyelinase |
| Antigen Species | Human |
| Gene Name | SMPD1 |
| Gene ID | |
| Protein Name | Sphingomyelin phosphodiesterase |
| Uniprot ID | |
| Biology Area | Associated Proteins,Neurodegenerative disease,Lipid metabolism |
| Function | 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. |
| Molecular Weight | Theoretical: 64 kDa. Actual: 69 kDa. |
| Stability & Storage | Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles. |
| Transport | Shipping with blue ice. |
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