基因名：
HEXB
产品别名：
ENC-1AS； HEL-248； HEL-S-111； HEXB； hexosaminidase subunit beta； hexosaminidase subunit beta； beta-hexosaminidase subunit beta； HCC-7； N-acetyl-beta-glucosaminidase subunit beta； beta-N-acetylhexosaminidase subunit beta； beta-hexosaminidase beta-subunit； cervical cancer proto-oncogene 7 protein； epididymis luminal protein 248； epididymis secretory protein Li 111； epididymis secretory sperm binding protein； hexosaminidase B (beta polypeptide)； hexosaminidase subunit B； Beta氨基己糖苷酶beta亚基蛋白A链； Beta氨基己糖苷酶beta亚基蛋白B链； 氨基己糖苷酶Bβ(HEXb)；
背景信息：
Hexosaminidase B is the beta subunit of the lysosomal enzyme beta-hexosaminidase that, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Beta-hexosaminidase is composed of two subunits, alpha and beta, which are encoded by separate genes. Both beta-hexosaminidase alpha and beta subunits are members of family 20 of glycosyl hydrolases. Mutations in the alpha or beta subunit genes lead to an accumulation of GM2 ganglioside in neurons and neurodegenerative disorders termed the GM2 gangliosidoses. Beta subunit gene mutations lead to Sandhoff disease (GM2-gangliosidosis type II). [provided by RefSeq, Jul 2008].
