{"product_id":"new-product-394826","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":"Bioworld","offers":[{"title":"100ul \/ Rabbit \/ Human","offer_id":44092029403360,"sku":"BS1917","price":325.0,"currency_code":"KRW","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0601\/9330\/8896\/products\/bioWORLD-Logo-New_1617713956_6b436983-a135-4459-8dc1-822101744a16.png?v=1689711499","url":"https:\/\/rndmate.com\/products\/new-product-394826","provider":"알앤디메이트","version":"1.0","type":"link"}