Acid sphingomyelinase Antibody, ALEXA FLUOR 488
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Catalog number
bs-6318R-A488
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Price
Please ask
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Size
100ul
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Long name
Acid sphingomyelinase Antibody, ALEXA FLUOR 488 Conjugated
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Also known as
Anti-Acid sphingomyelinase PAb ALEXA FLUOR 488
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Category
Conjugated Primary Antibodies
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Conjugated with
ALEXA FLUOR® 488
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Host Organism
Rabbit (Oryctolagus cuniculus)
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Target Antigen
Acid sphingomyelinase
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Specificity
This is a highly specific antibody against Acid sphingomyelinase.
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Modification
Unmodified
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Modification Site
None
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Clonality
Polyclonal
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Clone
Polyclonal antibody
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Concentration
1ug per 1ul
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Source
This antibody was obtained by immunization of the host with KLH conjugated synthetic peptide derived from human Acid sphingomyelinase
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Gene ID Number
6609
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Tested applications
IF(IHC-P)
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Recommended dilutions
IF(IHC-P)(1:50-200)
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Crossreactivity
Human, Mouse, Rat
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Cross reactive species details
Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.
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Background of the antigen
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.
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Purification
Purified by Protein A.
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Storage conditions
Store this antibody in aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Keep refrigerated at 2 to 8 degrees Celcius for up to one year.
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Excitation emission
499nm/519nm
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Synonyms
Acid sphingomyelinase; ASM; ASM_HUMAN; aSMase; NPD; Smpd1; Sphingomyelin phosphodiesterase 1 acid lysosomal; Sphingomyelin phosphodiesterase.
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Properties
For facs or microscopy Alexa 1 conjugate. Alexa Fluor 488 has the same range to that of fluorescein isothiocyanate (FITC), yet the Anti-Acid sphingomyelinase has a very high photo stability. As a result of this photo stability, it has turned into an antibody for fluorescent microscopy and FACS FLOW cytometry. It is distinguished in the FL1 of a FACS-Calibur or FACScan. Also Alexa Fluor 488 is pH stable. If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies. ALEXA FLUOR they should be stored frozen at - 24°C for long term storage and for short term at + 5°C.
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Conjugation
Alexa Fluor
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French translation
anticorps
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Gene target
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Gene symbol
SMPDL3A, SMPD1
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Short name
Anti-Acid sphingomyelinase
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Technique
Antibody, antibodies against human proteins, antibodies for
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Isotype
Immunoglobulin G (IgG)
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Label
ALEXA FLUOR 488
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Alternative name
Acid sphingomyelinase Antibody, ALEXA FLUOR 488
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Alternative technique
antibodies
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Gene info
Gene info
MeSH Data
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Name
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Concept
Scope note:
Identification of proteins or peptides that have been electrophoretically separated by blot transferring from the electrophoresis gel to strips of nitrocellulose paper, followed by labeling with antibody probes.
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Tree numbers
- E05.196.401.143
- E05.301.300.096
- E05.478.566.320.200
- E05.601.262
- E05.601.470.320.200
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Qualifiers
ethics, trends, veterinary, history, classification, economics, instrumentation, methods, standards, statistics & numerical data
Product images
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