HEXB (E9X5S) Mouse Chimeric Monoclonal Antibody#87218,Cell Signaling Technology (CST),87218

β-hexosaminidase (Hex) is an important lysosomal enzyme system that degrades various cellular substrates, such as oligosaccharides, glycosaminoglycans, and glycolipids. It is encoded by two genes,HEXAandHEXB, respectively, and these subunits dimerize to form β-hexosaminidase A (HexA; αβ) and β-hexosaminidase B (HexB; ββ) (1). Loss-of-function mutations result in ganglioside (GM2) accumulation and progressive neurodegenerative diseases, such as Sandhoff disease (SD) and Tay-Sachs disease (TSD) (1). HexB knockout mice display, similarly to human patients, a near-normal phenotype at birth but quickly develop muscle weakness, rigidity, and motor deterioration, typically leading to death at approximately four months of age (2). It has been shown that loss of HEXB leads to reduction of early neuronal migration and differentiation in the embryonic cerebral cortices of these mice (3). Hex also plays an important role in cancer, being a new potential biomarker in laryngeal cancer. Furthermore, higher expression of HEXA and HEXB is associated with poor prognosis in glioblastoma multiforme (GBM) patients (1).Jia, M. et al. (2021)Front Oncol11, 685893.Kyrkanides, S. et al. (2012)J Neuroinflammation9, 186.Ogawa, Y. et al. (2020)Neurosci Res155, 12-19.Alternate NamesBeta-hexosaminidase subunit beta; Beta-N-acetylhexosaminidase subunit beta; Hexb; hexosaminidase B; Hexosaminidase subunit B; N-acetyl-beta-glucosaminidase subunit beta

Beta-hexosaminidase subunit beta; Beta-N-acetylhexosaminidase subunit beta; Hexb; hexosaminidase B; Hexosaminidase subunit B; N-acetyl-beta-glucosaminidase subunit beta