The anti-HEXB antibody is designed to detect human Hexosaminidase subunit beta (HEXB), a critical lysosomal enzyme subunit involved in the degradation of GM2 gangliosides and other glycosaminoglycans. This antibody is optimized for use in immunohistochemistry (IHC) and Western blotting (WB) applications.
HEXB is one of the subunits of the enzyme beta-hexosaminidase, a lysosomal hydrolase responsible for degrading GM2 gangliosides and other glycosaminoglycans. Along with the alpha subunit (encoded by HEXA), HEXB forms functional isoenzymes (HexA and HexB) that play essential roles in lysosomal catabolism. HEXB is expressed in various tissues, with particularly high activity in the central nervous system. Mutations in the HEXB gene are associated with Sandhoff disease, a lysosomal storage disorder characterized by the accumulation of GM2 gangliosides, leading to progressive neurodegeneration. The study of HEXB is critical for understanding the molecular mechanisms of lysosomal function, glycosaminoglycan metabolism, and their dysregulation in diseases such as Sandhoff disease and other neurodegenerative conditions.
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Regulatory Status |
RUO – Research Use Only |
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