Anti-NPC2 Antibody

The NPC2 protein is a small, soluble lysosomal glycoprotein that binds cholesterol with high affinity and transfers it to NPC1, the partner membrane protein disrupted in Niemann-Pick type C disease. Loss-of-function mutations in the NPC2 gene cause Niemann-Pick disease type C2, a rare lysosomal storage disorder characterized by progressive neurodegeneration, hepatosplenomegaly, and visceral lipid accumulation, which has made NPC2 a target of interest in studies of intracellular cholesterol trafficking, neurodegeneration, and lipid metabolism.

This unconjugated rabbit polyclonal reagent from Antibodies.com was raised against NPC2 and is supplied at 1 mg/ml in PBS with 0.1% sodium azide and 50% glycerol, packaged as a 100 µl vial. It cross-reacts with human, mouse, and rat samples and is validated for western blot, immunohistochemistry, and immunofluorescence workflows. Investigators commonly use it to probe endogenous NPC2 in patient-derived fibroblasts, mouse brain sections, and hepatic lysates, where the antibody recognizes the mature secreted form of the protein. For best stability, store at -20°C in working aliquots to limit freeze-thaw cycles, and protect from light during handling.

Used in cell biology, neuroscience, and lysosomal storage disorder research, this primary antibody supports fixed-tissue IHC imaging of cholesterol-loaded macrophages as well as quantitative western blot detection of NPC2 in fractionated lysosomal and endosomal samples.