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中文名稱：人神經節苷脂GM2活化劑(GM2A)酶聯免疫試劑盒
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貨號：CSB-EL009565HU
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規格：96T/48T
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價格：￥3600/￥2500
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其他：
產品詳情
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產品描述：人神經節苷脂GM2活化劑(GM2A)酶聯免疫試劑盒（CSB-EL009565HU）為雙抗夾心法ELISA試劑盒，定量檢測血清、血漿、尿液、組織勻漿、細胞裂解物樣本中的GM2A含量。GM2A 是一個重要靶點，它與 GM2 神經節苷脂代謝相關。其研究機制主要圍繞在輔助降解 GM2 神經節苷脂上。若 GM2A 功能異常，會導致 GM2 神經節苷脂蓄積，引發神經系統疾病，故圍繞其開展的研究對相關疾病治療意義重大。試劑盒檢測范圍為15.6 pg/mL-1000 pg/mL，本試劑盒適用于生命科學領域的基礎研究，例如探究脂質代謝異常相關疾病的分子機制、評估疾病模型中GM2A的表達動態變化，或用于神經生物學、細胞生物學實驗中分析特定干預條件對GM2A蛋白水平的影響，為研究該靶點在生理病理過程中的作用提供定量化檢測工具。本品僅用于科研，不用于臨床診斷，產品具體參數及操作步驟詳見產品說明書。
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別名：Cerebroside sulfate activator protein ELISA Kit; ganglioside GM2 activator ELISA Kit; Ganglioside GM2 activator isoform short ELISA Kit; Ganglioside GM2 activator precursor ELISA Kit; GM2 activator ELISA Kit; GM2 AP ELISA Kit; GM2 ganglioside activator ELISA Kit; GM2 ganglioside activator protein ELISA Kit; GM2-AP ELISA Kit; GM2A ELISA Kit; GM2AP ELISA Kit; OTTHUMP00000160619 ELISA Kit; SAP 3 ELISA Kit; SAP-3 ELISA Kit; SAP3 ELISA Kit; SAP3_HUMAN ELISA Kit; Shingolipid activator protein 3 ELISA Kit; Sphingolipid activator protein 3 ELISA Kit
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縮寫：GM2A
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Uniprot No.：
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種屬：Homo sapiens (Human)
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樣本類型：serum, plasma, urine, tissue homogenates, cell lysates
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檢測范圍：15.6 pg/mL-1000 pg/mL
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靈敏度：3.9 pg/mL
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反應時間：1-5h
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樣本體積：50-100ul
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檢測波長：450 nm
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研究領域：Metabolism
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測定原理：quantitative
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測定方法：Sandwich
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精密度：
Intra-assay Precision (Precision within an assay): CV%<8% Three samples of known concentration were tested twenty times on one plate to assess. Inter-assay Precision (Precision between assays): CV%<10% Three samples of known concentration were tested in twenty assays to assess. -
線性度：
To assess the linearity of the assay, samples were spiked with high concentrations of human GM2A in various matrices and diluted with the Sample Diluent to produce samples with values within the dynamic range of the assay. Sample Serum(n=4) 1:1 Average % 89 Range % 84-95 1:2 Average % 92 Range % 87-99 1:4 Average % 100 Range % 95-106 1:8 Average % 97 Range % 92-102 -
回收率：
The recovery of human GM2A spiked to levels throughout the range of the assay in various matrices was evaluated. Samples were diluted prior to assay as directed in the Sample Preparation section. Sample Type Average % Recovery Range Serum (n=5) 96 90-103 EDTA plasma (n=4) 90 84-96 -
標準曲線：
These standard curves are provided for demonstration only. A standard curve should be generated for each set of samples assayed. 
pg/ml OD1 OD2 Average Corrected 1000 2.043 2.050 2.047 1.921 500 1.619 1.684 1.652 1.526 250 1.105 1.200 1.153 1.027 125 0.683 0.703 0.693 0.567 62.5 0.403 0.421 0.412 0.286 31.2 0.296 0.322 0.309 0.183 15.6 0.241 0.235 0.238 0.112 0 0.123 0.129 0.126 -
數據處理：
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貨期：3-5 working days
引用文獻
產品評價
相關產品
靶點詳情
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功能：The large binding pocket can accommodate several single chain phospholipids and fatty acids, GM2A also exhibits some calcium-independent phospholipase activity. Binds gangliosides and stimulates ganglioside GM2 degradation. It stimulates only the breakdown of ganglioside GM2 and glycolipid 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. Has cholesterol transfer activity.
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基因功能參考文獻：
- Review of GM2A mutations causing GM2 activator protein deficiency and GM2 gangliosidosis-AB variant. PMID: 27402091
- Studies indicate that sphingolipid activator proteins (SAPs) and anionic lipids are essential stimulators to reach physiological rates of lysosomal sphingolipid degradation. PMID: 27157270
- this study has established the potential role of GM2A in breast cancer progression PMID: 27002480
- Mobilization of membrane lipids by GM2AP was also inhibited in the presence of cholesterol or SM, as revealed by surface plasmon resonance studies PMID: 26175473
- Gene polymorphisms of MD2 and GM2A were associated with the occurrence or severity of neonatal necrotizing enterocolitis. PMID: 25816011
- In vitro assays with the isolated H1 or H2 homodimers (beta-alpha hybrid construct of beta-hexosaminidase A subunits) confirmed that neither was capable of human GM2AP-dependent hydrolysis of GM2 ganglioside. PMID: 23483939
- impact of GM2AP on glucose metabolism PMID: 21784073
- Treatment of meniscal explants with IL-1RA inhibited the expression of many catabolic genes following a single bout of high dynamic strain. PMID: 21331553
- these results provide novel insights into the physiological functions of GM2AP in obesity. PMID: 21036149
- GM2 activator protein exerts strong and broad inhibitory effects on the hydrolysis of phospholipids carried out by plant and microbial phospholipases D PMID: 12576516
- Two new structures of GM2-AP with bound lipids, showing two different lipid-binding modes within the apolar pocket PMID: 12909021
- elucidation of mode of action on gangioside GM2 PMID: 14728689
- alpha-subunit loop structure is required for GM2 activator protein binding by beta-hexosaminidase A PMID: 15485660
- glycosphingolipids, particularly GM2, form a complex with CD82, and this complex interacts with Met and thereby inhibits HGF-induced Met tyrosine kinase activity, as well as integrin to Met cross-talk PMID: 17215249
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相關疾病：GM2-gangliosidosis AB (GM2GAB)
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亞細胞定位：Lysosome.
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數據庫鏈接：
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