Acid sphingomyelinase Antibody, Biotin Conjugated

Catalog number:	bs-6318R-Biotin Buy Now
Price:	2735 €
Supplier:	MBS Recombinant Proteins
Product name:	Acid sphingomyelinase Antibody, Biotin Conjugated
Quantity:	100ug
Related search:	antibody sphingomyelinase antibodies sphingomyelin niemannpick types severe

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Long name:	Biotin Conjugated, Acid sphingomyelinase Antibody
Category:	Conjugated Primary Antibodies
Conjugation:	Biotinylated
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:	KLH conjugated synthetic peptide derived from human Acid sphingomyelinase
Gene ID number:	6609
Tested applications:	IHC-P, WB
Recommended dilutions:	IHC-P(1:100-500), WB(1:100-1000)
Crossreactivity:	Mouse, Rat, Human
Crossreactive species details:	not every possible cross-reactivity is known, Due to limited amount of testing and knowledge
Antigen background:	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:	This antibody was purified via Protein A
Storage conditions:	50% glycerol and 0, Store refrigerated at 2 to 8 degrees Celcius for up to 1 year, 09% sodium azide, Keep the antibody in an aqueous buffered solution containing 1% BSA
Synonyms:	ASM, ASM_HUMAN, NPD, Smpd1, Sphingomyelin phosphodiesterase, Sphingomyelin phosphodiesterase 1 acid lysosomal, aSMase, Acid sphingomyelinase
Properties:	Avidin and Streptavidin bind to the small biotin and are couple to HRP or AP for ELISA, Biotin conjugates can be detected by horseradish peroxidase, To break the streptavidin Biotin bond we suggest to use a 6 molar guanidine HCl solution with acidity of pH 1, alkaline phosphatase substrates or anti biotin conjugated antibodies, 6, C, C for long term storage and for short term at + 5°, If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies they should be stored frozen at - 24°
Gene target:	Acid sphingomyelinase Conjugated
Short name:	Conjugated, Acid sphingomyelinase Antibody
Technique:	antibodies against human proteins, antibodies for, antibody Conjugates, Antibody
Label:	Biotin
Alternative name:	biotinilated coupled, Acid sphingomyelinase (Antibody to)
Alternative technique:	antibodies

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