Analytical Data
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基因名
GM2A
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简介
Ganglioside GM2 activator (GM2A) is a small glycolipid transport protein which acts as a substrate specific co-factor for the lysosomal enzyme beta-hexosaminidase A. GM2A catalyzes the degradation of the ganglioside GM2 as well as affects lipid storage and neuromuscular process controlling balance. GM2A Protein, Human (HEK293, His) is the recombinant human-derived GM2A protein, expressed by HEK293 , with C-6*His labeled tag.
- Application
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别名
Ganglioside GM2 activator; Cerebroside sulfate activator protein; GM2-AP; Sphingolipid activator protein 3; SAP-3
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种属
Human
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表达系统
HEK293
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标签
C-6*His
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纯度
Greater than 90% as determined by SDS-PAGE.
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蛋白编号
AAH09273.1
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表达区间
S32-I193
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蛋白长度
Full Length of GM2-AP
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分子量
19-22 kDa
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内毒素
< 1.0 EU per μg protein as determined by the LAL method.
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性状
Freeze-dried powder
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缓冲液
PBS, pH7.4, containing 0.01% SKL, 1mM DTT, 5% Trehalose and Proclin300.
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复溶方法
Reconstitute in ddH2O to a concentration of 0.1-0.5 mg/mL. Do not vortex.
- 个性化定制
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稳定性测试
The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37℃ for 48h, and no obvious degradation and precipitation were observed. The loss rate isless than 8% within the expiration date under appropriate storage condition.
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保存条件 & 期限
Samples are stable for up to twelve months from date of receipt at -20℃ to -80℃. Store it under sterile conditions at -20℃ to -80℃. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.
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运输条件
In general, recombinant proteins are supplied as lyophilized powder and shipped at ambient temperature. For bulk packages, the proteins are provided as frozen liquid and shipped with blue ice, unless otherwise requested by the customer.
Quality inspection process
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Protein Description
GM2A, or GM2 activating protein, plays a crucial role in the metabolism of GM2 gangliosides, which are glycosphingolipids found in the nervous system. The study of GM2A is particularly important due to its association with GM2 ganglioside accumulation in lysosomal storage disorders, such as GM2 gangliosidoses, which include Tay-Sachs disease and Sandhoff disease. These conditions are characterized by the deficiency of specific hexosaminidase enzymes, leading to harmful levels of GM2 in the body, particularly affecting neuronal function and resulting in severe neurological symptoms. Understanding the structure and function of GM2A can provide insights into the molecular mechanisms underlying these disorders, potentially facilitating the development of therapeutic strategies. Researchers have focused on GM2A's role as a cofactor in the enzymatic degradation of GM2 gangliosides, enhancing the activity of hexosaminidase enzymes. Furthermore, GM2A's interactions with other cellular components and lipid membranes are of significant interest, as they may influence the lysosomal pathway and ganglioside metabolism. Advances in recombinant protein technology have enabled the production of GM2A for in vitro studies, allowing for detailed investigations of its biochemical properties, stability, and functionality. Such research is vital for elucidating the pathophysiology of GM2-related diseases and may ultimately lead to innovative treatment methods, including enzyme replacement therapies or gene therapies aimed at restoring normal metabolic function in affected individuals. As our understanding of GM2A and its mechanisms improves, it holds promise for addressing the challenges posed by GM2 gangliosidoses and enhancing patient outcomes.
