Rabbit Anti-Acid sphingomyelinase Polyclonal Antibody
DATASHEET
Host:Rabbit
Target Protein:Acid sphingomyelinase
IR:Immunogen Range:201-300/629
Clonality:Polyclonal
Isotype:IgG
Entrez Gene:6609
Swiss Prot:P17405
Source:KLH conjugated synthetic peptide derived from human Acid sphingomyelinase:201-300/629
Purification:affinity purified by Protein A
Storage:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Background: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.
Size:50ul
Concentration:1mg/ml
Applications:WB(1:500-2000)
ELISA(1:5000-10000)
IHC-P(1:100-500)
IHC-F(1:100-500)
Flow-Cyt(2ug/Test)
ICC(1:100-500)
IF(1:100-500)
Cross Reactive Species:Human
Mouse
Rat
Dog
Pig
Cow
Rabbit
本品仅供科研使用。不能用于人和动物治疗等其它临床诊断用！
关键字： Acid sphingomyelinas;Acid sphingomyelinas;ASM;aSMase;NPD;Smpd1; Sphingomyelin phosp;ASM_HUMAN;
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