Seyrantepe, Volkan2020-07-252020-07-2520161096-71921096-7206https://doi.org/10.1016/j.ymgme.2015.12.434https://hdl.handle.net/11147/946212th Annual WORLD Symposium -- FEB 29-MAR 04, 2016 -- San Diego, CATay-Sachs disease is a severe lysosomal disorder caused by mutations in the HEXA gene coding for α subunit of lysosomal βhexosaminidase A which converts GM2 to GM3 ganglioside. HexA-/-mice, depleted of β-hexosaminidase A gene, remains asymptomatic to 1 year of age, owing to the ability of these mice to catabolise stored GM2 ganglioside via sialidase(s) removing sialic acid into glycolipid GA2 which further processed by β-Hexosaminidase B, thereby bypassing the HexA defect.eninfo:eu-repo/semantics/openAccessConference Object10.1016/j.ymgme.2015.12.434