{"product_id":"new-product-272862","title":"Hexb (S516) polyclonal antibody","description":"\u003cp\u003eHexosaminidase B (HEXB), also designated beta-hexosaminidase B, is a Hexosaminidase B (HEXB), also designated b-hexosaminidase B, is a tetramer of two b-A and two b-B chains and is found in the lysosomes of cells. Sandhoff disease (SD), also known as GM2-gangliosidosis type II, is caused by mutations in the HEXB gene that affect the b subunit. These mutations disrupt the activity of HEXB and HEXA, which prevents the breakdown of GM2 ganglioside, a fatty material found in the brain, therby rendering both the HEXA and HEXB enzymes deficient. SD is a rare autosomal recessive disorder characterized by an accumulation of GM2 ganglioside, which causes progressive destruction of the central nervous system. Sandhoff disease is similar to Tay-Sachs disease, which is caused by mutations in the HEXA gene, although SD is more severe.\u003c\/p\u003e","brand":"ABCbiolab","offers":[{"title":"100ul \/ Rabbit \/ Human","offer_id":45803259068696,"sku":"ABCBW00952","price":325.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0590\/5652\/1400\/products\/758-7580448_monoclonal-antibody-png-transparent-png_08b7a39f-e5d6-4426-991b-f9985094760c.png?v=1689733708","url":"https:\/\/danabiosci.com\/products\/new-product-272862","provider":"Dana Bioscience","version":"1.0","type":"link"}