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Acid sphingomyelinase Antibody, APC Conjugated

Applications

  • WB
  • IF(IHC-P)
  • IF(IHC-F)
  • IF(ICC)

Reactivity

  • Human
  • Mouse
  • Rat

Predicted Reactivity

  • Dog
  • Cow
  • Pig
  • Rabbit
Overview
Catalog # bs-6318R-APC
Product Name Acid sphingomyelinase Antibody, APC Conjugated
Applications WB, IF(IHC-P), IF(IHC-F), IF(ICC)
Reactivity Human, Mouse, Rat
Predicted Reactivity Dog, Cow, Pig, Rabbit
Specifications
Conjugation APC
Host Rabbit
Source KLH conjugated synthetic peptide derived from human Acid sphingomyelinase
Immunogen Range 201-300/629
Clonality Polyclonal
Isotype IgG
Concentration 1ug/ul
Purification Purified by Protein A.
Storage Buffer Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage Condition Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
Target
Gene ID 6609
Swiss Prot P17405
Subcellular location Cytoplasm
Synonyms Acid sphingomyelinase; ASM; ASM_HUMAN; aSMase; NPD; Smpd1; Sphingomyelin phosphodiesterase 1 acid lysosomal; Sphingomyelin phosphodiesterase.
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.
Application Dilution
WB 1:300-5000
IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200