Rabbit Polyclonal LARGE Antibody

The LARGE antibody is a crucial tool in studying the function of the LARGE protein (encoded by the *LARGE1* gene), a glycosyltransferase critical for post-translational modification of α-dystroglycan (α-DG). α-DG, a key component of the dystrophin-glycoprotein complex, requires extensive glycosylation to interact with extracellular matrix proteins like laminin, ensuring muscle membrane stability and neuronal development. Mutations in *LARGE1* disrupt this glycosylation, leading to congenital muscular dystrophies (e.g., Walker-Warburg syndrome) and brain abnormalities. Researchers developed LARGE antibodies to detect and quantify LARGE protein expression, assess glycosylation status, and investigate disease mechanisms. These antibodies are widely used in techniques like Western blotting, immunohistochemistry, and immunofluorescence to visualize LARGE localization and function in tissues or cell models. Studies also utilize LARGE antibodies to explore therapeutic strategies, such as gene therapy or small molecules aimed at restoring α-DG glycosylation. Notably, LARGE overexpression has been shown to hyperglycosylate α-DG, even in some secondary dystroglycanopathies, highlighting its therapeutic potential. The antibody’s role extends to basic research on glycan biosynthesis and neuromuscular biology, making it indispensable for understanding and treating glycosylation-related disorders.