Mouse Monoclonal NPC1 Antibody

NPC1 (Niemann-Pick disease type C1) antibodies are critical tools in studying the molecular mechanisms underlying Niemann-Pick type C (NPC), a rare lysosomal storage disorder caused by mutations in the NPC1 or NPC2 genes. The NPC1 protein, located in lysosomal and endosomal membranes, facilitates cholesterol and lipid trafficking. Dysfunctional NPC1 leads to abnormal accumulation of cholesterol and glycosphingolipids in lysosomes, resulting in progressive neurodegeneration and visceral organ damage. Antibodies targeting NPC1 are widely used in research to detect protein expression, localization, and functional alterations in cellular and animal models of NPC disease. They help elucidate disease pathology, evaluate therapeutic interventions (e.g., cyclodextrins, gene therapy), and validate biomarkers. Additionally, NPC1 antibodies have diagnostic applications, aiding in confirming NPC1 mutations through immunohistochemistry or Western blotting in patient-derived cells. Recent studies also explore NPC1’s role beyond NPC disease, including its involvement in viral entry (e.g., Ebola virus) and cancer metabolism. However, challenges remain in developing high-specificity antibodies due to NPC1’s complex transmembrane structure. Ongoing efforts focus on optimizing antibody performance for both basic research and clinical diagnostics, contributing to improved understanding and management of NPC-related disorders.