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.
Alternate Names:
Acid sphingomyelinase; ASM; ASM_Hu; aSMase; NPD; Smpd1; Sphingomyelin phosphodiesterase 1 acid lysosomal; Sphingomyelin phosphodiesterase.
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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