Name : Anti-Acid sphingomyelinase, ALEXA Fluor 594
Supplier : BIOSS POLYCLONAL ANTIBODIES
Price :489
SKU : GEN7566889892
| Type | Conjugated Primary Antibody |
| Conjugated with | 594, ALEXA FLUOR® |
| Host organism | Rabbit (Oryctolagus cuniculus) |
| Target Protein/Peptide | Acid sphingomyelinase |
| Specificity | This antibody reacts specifically with Acid sphingomyelinase |
| Modification | No modification has been applied to this antibody |
| Modification site | None |
| Clonality | Polyclonal Antibody |
| Clone | Polyclonal Antibodies |
| Concentration | 1ug per 1ul |
| Subcellular locations | N/A |
| Antigen Source | KLH conjugated synthetic peptide derived from human Acid sphingomyelinase |
| Gene ID | 6609 |
| Swiss Prot | N/A |
| Applications | IF(IHC-P) |
| Applications with corresponding dilutions | IF(IHC-P)(1:50-200) |
| Cross reactive species | Mouse (Mus musculus), Rat (Rattus norvegicus), Human (Homo sapiens) |
| Cross Reactive Species details | However, note that due to limited knowledge it is impossible to predict with 100% guarantee that the antibody does not corss react with any other species, No significant cross reactivity has been observed for this antibody for the tested species |
| Background information | A phenotypic continuum exists between type A (basic neurovisceral) and type B (purely visceral) forms of Niemann-Pick disease, Also has phospholipase C activities toward 1, Clinical features are variable, Isoform 2 and isoform 3 have lost catalytic activity, 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, Niemann-Pick disease type A is a primarily neurodegenerative disorder characterized by onset within the first year of life, The severe neurological disorders and pulmonary infections lead to an early death, also known as Niemann-Pick disease classical infantile form, and severe neurologic symptoms, and the intermediate types encompass a cluster of variants combining clinical features of both types A and B, digestive disorders, failure to thrive, leading to cell death, major hepatosplenomegaly, mental retardation, often around the age of four, 2-diacylglycerolphosphocholine and 1, 2-diacylglycerolphosphoglycerol, Converts sphingomyelin to ceramide, Involvement in disease: Defects in SMPD1 are the cause of Niemann-Pick disease type A (NPDA) |
| Purification method | Purified by Protein A |
| Storage | 50% glycerol and 0, Store at 4°, 09% sodium azide, C for 12 months, Water buffered solution containing 100ug/ml BSA |
| Excitation emission | 590nm/617nm |
| Synonyms | ASM, ASM_HUMAN, NPD, Smpd1, Sphingomyelin phosphodiesterase, Sphingomyelin phosphodiesterase 1 acid lysosomal, aSMase, Acid sphingomyelinase |
| Also known as | Acid sphingomyelinase Antibody |
| Other name | Anti-Acid sphingomyelinase |
| Advisory | For antibodies that are in liquid form or reconstituted lyophilized antibodies small amounts could become entrapped on the seal or the walls of the tube, Prior to use briefly centrifuge the vial to gather all the solution on the bottom, specificity and sensitivity, thus reducing its reactivity, Avoid freeze/thaw cycles as they may denaturate the polypeptide chains of the antibody |
| Properties | For facs or microscopy Alexa 1 conjugate |
| Conjugation | Alexa Fluor |
| Gene target | Acid sphingomyelinase |
| Short name | Fluor 594, Anti-Acid sphingomyelinase |
| Label | ALEXA |
| Alternative name | ALEXA Fluor 594, antibody to-Acid sphingomyelinase |