神经鞘脂激活蛋白3抗体
规格：1尘驳/1尘濒
英文名： GM2A
别名： Cerebroside sulfate activator protein; Ganglioside GM2 activator isoform short; Ganglioside GM2 activator precursor; GM2 AP; GM2 ganglioside activator; GM2 ganglioside activator protein; GM2-AP; GM2A;
分子量： 17/18kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human GM2A/SAP
交叉反应：贬耻尘补苍,
细胞定位：细胞浆
神经鞘脂激活蛋白3抗体产物介绍：background: This gene encodes a small glycolipid transport protein which acts as a substrate specific co-factor for the lysosomal enzyme beta-hexosaminidase A. Beta-hexosaminidase A, together with GM2 ganglioside activator, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Mutations in this gene result in GM2-gangliosidosis type AB or the AB variant of Tay-Sachs disease. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Nov 2009]. Function: Binds gangliosides and stimulates ganglioside GM2 degradation. It stimulates only the breakdown of ganglioside GM2 and glycolipid神经鞘脂激活蛋白3抗体 GA2 by beta-hexosaminidase A. 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. Subcellular Location: Lysosome. Post-translational modifications: The serines in positions 32 and 33 are absent in 80% of the sequenced protein. DISEASE: Defects in GM2A are the cause of GM2-gangliosidosis type AB (GM2GAB) [MIM:272750]; also known as Tay-Sachs disease AB variant. GM2-gangliosidosis is an autosomal recessive 神经鞘脂激活蛋白3抗体lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. GM2GAB is characterized by GM2 gangliosides accumulation in the presence of both hexosaminidase A and B. Database links: UniProtKB/Swiss-Prot: P17900.4 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
神经鞘脂激活蛋白3抗体产物应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 神经生物学 细胞类型标志物 新陈代谢
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid