The RNF213 antibody is a crucial tool for studying the RNF213 protein, a large E3 ubiquitin ligase implicated in vascular diseases, particularly Moyamoya disease (MMD). RNF213. encoded by the *RNF213* gene on chromosome 17q25.3. regulates cellular processes like angiogenesis, inflammation, and lipid metabolism. Its genetic variants, especially the p.R4810K mutation prevalent in East Asian populations, are strongly associated with MMD, a cerebrovascular disorder characterized by stenosis of intracranial arteries. RNF213 antibodies enable detection and analysis of this protein's expression, localization, and interactions in research models. These antibodies are typically developed against specific epitopes, such as the N-terminal or C-terminal regions, to ensure specificity given the protein's large size (~5.200 amino acids). Studies using RNF213 antibodies have revealed its role in endothelial cell dysfunction, vascular remodeling, and immune responses. Additionally, they aid in exploring how mutations alter protein stability, ubiquitination activity, or subcellular distribution, contributing to disease mechanisms. Commercial RNF213 antibodies are widely used in techniques like Western blotting, immunohistochemistry, and immunofluorescence, though variability in antibody performance across studies highlights the need for validation. Ongoing research aims to clarify RNF213's pathophysiology and therapeutic potential, positioning its antibody as a vital reagent in vascular and neurological research.