酸性神经鞘磷脂酶抗体

ASM/Acid sphingomyelinase Antibody

Cat#：PL0300576

Isotype:Rabbit Polyclonal IgG

Immunogen：Peptide derived from Hu Acid sphingomyelinase.

Purification: Immunogen affinity purified

Storage Buffer: PBS, pH 7.4(1%BSA and 0.1% Sodium azide)

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.

Specificity Reaction： Hu, Mo, Rat, Dog, Pig, Cow

Application Notes:WB: 1:100-500 E: 1:500-1000 IHC: 1:100-500 IF: 1:100-500

Storage ：

Store at –20ºC for one year. Avoid repeated freeze-thaw cycles. At 4ºC for 2-3 weeks after reconstituted.

This product for research use only, not for use in humans, therapeutic or diagnostic procedures.

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