Acid sphingomyelinase Antibody

Catalog number:	bs-6318R Buy Now
Price:	2735 €
Supplier:	MBS Recombinant Proteins
Product name:	Acid sphingomyelinase Antibody
Quantity:	100ug
Other quantities:	100ug 536€
Related search:	antibody sphingomyelinase sphingomyelin disease phosphodiesterase synonyms features

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Long name:	Acid sphingomyelinase Antibody
Also known as:	Acid sphingomyelinase Antibody
Category:	Primary Antibodies
Conjugation:	Unconjugated
Target Antigen:	Acid sphingomyelinase
Specificity:	This is a highly specific antibody against Acid sphingomyelinase
Modification(s):	None
Modification site(s):	Unmodified antibody
Clonality:	Polyclonal antibody
Clone number:	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), IHC-P, WB
Recommended dilutions:	IF(IHC-P)(1:50-200), IHC-P(1:100-500), WB(1:100-1000)
Cross reactivity:	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 target 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 method:	This antibody was purified via Protein A
Storage conditions:	50% glycerol and 0, Store at -20°, 09% sodium azide, C for up to 1 year, Keep the antibody in aqueous buffered solution containing 1% BSA
Synonym names:	ASM, ASM_HUMAN, NPD, Smpd1, Sphingomyelin phosphodiesterase, Sphingomyelin phosphodiesterase 1 acid lysosomal, aSMase, Acid sphingomyelinase
Properties:	C, C for long term storage and for short term at + 5°, If you buy Antibodies supplied by Bioss Primary Unconjugated Antibodies they should be stored frozen at - 24°
Gene target:	Acid sphingomyelinase
Short name:	Acid sphingomyelinase Antibody
Technique:	antibodies against human proteins, antibodies for, Antibody
Alternative name:	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:	ASM
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

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