Product Name :Niemann Pick C1 Recombinant Rabbit mAb (KU)
Reactivity :Human, Mouse, Rat
Applications :WB,IHC,ICC/IF,FC
Application_all :WB: 1:2000<br />IHC: 1:100<br />ICC/IF: 1:100<br />FC: 1:50
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 :Store at -20°C. Supplied in 50mM Tris-Glycine(pH 7.4), 0.15M NaCl, 40%Glycerol, 0.01% sodium azide and 0.05% BSA. Stable for 12 months from date of receipt.
Purification&Purity :Affinity Purification
Storage&Stability :Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Note :For research use only, not for use in diagnostic procedure.
Immunogen :A synthetic peptide of human Niemann Pick C1
Modification :Unmodification
