Acid sphingomyelinase Polyclonal Antibody | BS65948

Acid sphingomyelinase Polyclonal Antibody | BS65948 | Gentaur UK, US & Europe Distribution

Host: Rabbit

Reactivity: Human,Mouse,Rat,Dog,Pig,Cow,Rabbit,

Application: FCM,

Application Range: Flow-Cyt=2ug/Test

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

Storage & Stability: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

Specificity: Acid sphingomyelinase Polyclonal Antibody detects endogenous levels of Acid sphingomyelinase protein.

Molecular Weight: 64kDa

Note: For research use only, not for use in diagnostic procedure.

Alternative Names: Acid sphingomyelinase; ASM; ASM_HUMAN; aSMase; NPD; Smpd1; Sphingomyelin phosphodiesterase 1 acid lysosomal; Sphingomyelin phosphodiesterase.

Immunogen: KLH conjugated synthetic peptide derived from human Acid sphingomyelinase:201-300/629

Conjugate: Unconjugated

Modification: Unmodification

Purification & Purity: affinity purified by Protein A

Pathway: