Rabbit Polyclonal BSCL2(N-term) Antibody

The BSCL2 (N-term) antibody is designed to detect the N-terminal region of the BSCL2 protein, encoded by the Berardinelli-Seip congenital lipodystrophy type 2 (BSCL2) gene. This gene is critical for lipid metabolism and adipocyte differentiation, with mutations linked to congenital generalized lipodystrophy (CGL), a rare disorder characterized by near-complete absence of adipose tissue and metabolic complications like insulin resistance. BSCL2 encodes seipin, an endoplasmic reticulum (ER)-resident protein involved in lipid droplet formation, adipogenesis, and neuronal maintenance. Dysfunctional seipin disrupts lipid storage and ER stress pathways, contributing to metabolic and neurodegenerative diseases. The antibody targets the N-terminal domain, which is crucial for seipin's oligomerization and interaction with lipid synthesis regulators. Researchers use this antibody in Western blotting, immunohistochemistry, and immunofluorescence to study seipin expression, localization, and mutations in cellular or tissue models. It also aids in diagnosing BSCL2-related disorders and investigating molecular mechanisms underlying lipodystrophies, neurodegenerative conditions (e.g., seipinopathy), and metabolic syndromes. Its specificity for the N-terminal region ensures accurate detection of full-length seipin and potential truncation variants.