| Long name: |
ALEXA FLUOR 488 Conjugated, Acid sphingomyelinase Antibody |
| Also known as: |
Anti-Acid sphingomyelinase PAb ALEXA FLUOR 488 |
| Category: |
Conjugated Primary Antibodies |
| Conjugated with: |
488, ALEXA FLUOR® |
| Host Organism: |
Rabbit (Oryctolagus cuniculus) |
| Target Antigen: |
Acid sphingomyelinase |
| Specificity: |
This is a highly specific antibody against Acid sphingomyelinase |
| Modification: |
Unmodified |
| Modification Site: |
None |
| Clonality: |
Polyclonal |
| Clone: |
Polyclonal antibody |
| Concentration: |
1ug per 1ul |
| Source: |
This antibody was obtained by immunization of the host with KLH conjugated synthetic peptide derived from human Acid sphingomyelinase |
| Gene ID Number: |
6609 |
| Tested applications: |
IF(IHC-P) |
| Recommended dilutions: |
IF(IHC-P)(1:50-200) |
| Crossreactivity: |
Mouse, Rat, Human |
| Cross-reactive species details: |
not every possible cross-reactivity is known, Due to limited amount of testing and knowledge |
| Background of the antigen: |
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: |
Purified by Protein A |
| Storage conditions: |
50% glycerol and 0, Keep refrigerated at 2 to 8 degrees Celcius for up to one year, 09% sodium azide, Store this antibody in aqueous buffered solution containing 1% BSA |
| Excitation emission: |
499nm/519nm |
| Synonyms: |
ASM |
| Properties: |
ALEXA FLUOR they should be stored frozen at - 24°, Alexa Fluor 488 has the same range to that of fluorescein isothiocyanate (FITC), Also Alexa Fluor 488 is pH stable, As a result of this photo stability, If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies, It is distinguished in the FL1 of a FACS-Calibur or FACScan, has a very high photo stability, it has turned into an antibody for fluorescent microscopy and FACS FLOW cytometry, yet the Acid sphingomyelinase Antibody, C, C for long term storage and for short term at + 5°, For facs or microscopy Alexa 1 conjugate |
| Conjugation: |
Alexa Fluor |
| Gene target: |
Acid sphingomyelinase |
| Short name: |
Acid sphingomyelinase Antibody |
| Technique: |
antibodies against human proteins, antibodies for, Antibody |
| Label: |
ALEXA FLUOR 488 |
| Alternative name: |
ALEXA FLUOR 488, Acid sphingomyelinase (Antibody to) |
| Alternative technique: |
antibodies |
| Identity: |
11120 |
| Gene: |
SMPD1 |
More about : SMPD1 |
| Long gene name: |
sphingomyelin phosphodiesterase 1 |
| Synonyms gene name: |
acid lysosomal , sphingomyelin phosphodiesterase 1 |
| Synonyms name: |
acid sphingomyelinase |
| Locus: |
11p15, 4 |
| Discovery year: |
1986-01-01 |
| GenBank acession: |
AB209775 |
| Entrez gene record: |
6609 |
| Pubmed identfication: |
1711683 |
| RefSeq identity: |
NM_000543 |
| Havana BLAST/BLAT: |
OTTHUMG00000165453 |