Acid sphingomyelinase Antibody
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Catalog number
bs-6318R
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Price
Please ask
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Size
0.1ml
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Long name
Acid sphingomyelinase Antibody
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Also known as
Acid sphingomyelinase Antibody
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Category
Primary Antibodies
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Conjugation
Unconjugated
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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 s
None
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Modification site s
Unmodified antibody
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Clonality
Polyclonal antibody
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Clone number
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
WB, IHC-P, IF(IHC-P)
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Recommended dilutions
WB(1:100-1000), IHC-P(1:100-500), IF(IHC-P)(1:50-200)
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Cross reactivity
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 target 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 method
This antibody was purified via Protein A.
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Storage conditions
Keep the antibody in aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Store at -20°C for up to 1 year.
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Synonym names
Acid sphingomyelinase; ASM; ASM_HUMAN; aSMase; NPD; Smpd1; Sphingomyelin phosphodiesterase 1 acid lysosomal; Sphingomyelin phosphodiesterase.
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Properties
If you buy Antibodies supplied by Bioss Primary Unconjugated Antibodies they should be stored frozen at - 24°C for long term storage and for short term at + 5°C.
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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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Host
Rabbit (Oryctolagus cuniculus)
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Isotype
Immunoglobulin G (IgG)
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Alternative name
Acid sphingomyelinase Antibody
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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
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