背景:GM2A (GM2 ganglioside activator), is a lipid transfer protein which belongs to the ML domain family.
GM2A can accommodate several single chain phospholipids and fatty acids.
It also exhibits some calcium-independent phospholipase activity.
GM2A binds gangliosides and stimulates ganglioside GM2 degradation.
It stimulates only the breakdown of ganglioside GM2 and glycolipid GA2 by beta-hexosaminidase A.
GM2A acts as a substrate specific co-factor for the lysosomal enzyme β-hexosaminidase A.
β-hexosaminidase A, together with GM2 ganglioside activator, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines.
It extracts single GM2 molecules from membranes and presents them in soluble form to beta-hexosaminidase A for cleavage of N-acetyl-D-galactosamine and conversion to GM3.
Defects in GM2A are the cause of GM2-gangliosidosis type AB (GM2GAB), also known as Tay-Sachs disease AB variant.
仕様
サイズ:50ug
種由来:Human
発現宿主:Baculovirus-Insect Cells
純度:>96 % as determined by reducing SDS-PAGE.
エンドトキシン濃度:<1.0 EU per μg of the protein as determined by the LAL method.
アクセッションNo.:AAA35907.1
アミノ酸配列:Met 1-Ile 193
遺伝子構造:A DNA sequence encoding the human GM2A (AAA35907.1) (Met 1-Ile 193) was fused with a polyhistidine tag at the C-terminus.
別名(遺伝子名):Ganglioside GM2 activator;Cerebroside sulfate activator protein;GM2-AP;Sphingolipid activator protein 3;SAP-3
保存条件:Generally, lyophilized proteins are stable for up to 12 months when stored at -20 to -80℃. Reconstituted protein solution can be stored at 4-8℃ for 2-7 days. Aliquots of reconstituted samples are stable at < -20℃ for 3 months.