The ARSB antibody targets the enzyme Arylsulfatase B (ARSB), also known as N-acetylgalactosamine-4-sulfatase, which plays a critical role in lysosomal catabolism. ARSB is responsible for hydrolyzing sulfate groups from glycosaminoglycans (GAGs), specifically dermatan sulfate and chondroitin sulfate, during their degradation. Deficiencies in ARSB activity due to genetic mutations lead to the lysosomal storage disorder mucopolysaccharidosis type VI (MPS VI or Maroteaux-Lamy syndrome), characterized by skeletal abnormalities, organomegaly, and impaired growth.
ARSB antibodies are essential tools in biomedical research and diagnostics. They are used to detect ARSB protein expression in tissues or cell lines via techniques like Western blotting, immunohistochemistry, and immunofluorescence. These applications help assess ARSB levels in MPS VI patients, aiding diagnosis and therapeutic monitoring. Additionally, ARSB is studied beyond lysosomal disorders due to emerging roles in cellular processes such as inflammation, fibrosis, and cancer. For example, altered ARSB expression has been linked to tumor progression and metastasis in certain cancers, possibly through interactions with extracellular matrix components.
Research-grade ARSB antibodies are typically validated for specificity and sensitivity, enabling precise investigation of its enzymatic activity and regulatory mechanisms. Their utility extends to exploring ARSB's involvement in sulfate metabolism and its broader impact on cellular signaling pathways.