Product Name :Niemann Pick C1 Recombinant Rabbit mAb
Reactivity :Human,Mouse,Rat
Applications :WB,IHC-P,ICC/IF,FC
Application_all :WB: 1:1000-1:5000 ; IHC-P: 1:50-1:100 ; ICC/IF: 1:50-1:100 ; FC: 1:100-1:200
Background :This gene encodes a large protein that resides in the limiting membrane of endosomes and lysosomes and mediates intracellular cholesterol trafficking via binding of cholesterol to its N-terminal domain. It is predicted to have a cytoplasmic C-terminus, 13 transmembrane domains, and 3 large loops in the lumen of the endosome - the last loop being at the N-terminus. This protein transports low-density lipoproteins to late endosomal/lysosomal compartments where they are hydrolized and released as free cholesterol. Defects in this gene cause Niemann-Pick type C disease, a rare autosomal recessive neurodegenerative disorder characterized by over accumulation of cholesterol and glycosphingolipids in late endosomal/lysosomal compartments.[provided by RefSeq, Aug 2009]
Product :1mg/ml in 50mM Tris-Glycine(pH 7.4), 0.15M NaCl, 40% Glycerol, 0.01% sodium azide and 0.05% BSA
Purification&Purity :Affinity Purification
Storage&Stability :Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Specificity :The antibody detects endogenous levels of it
Note :For research use only, not for use in diagnostic procedure.
Immunogen :A synthetic peptide of human Niemann Pick C1
Modification :Unmodification
