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全部產(chǎn)品分類
品牌 |
Leading Biology | 貨號(hào) |
LM20352 |
產(chǎn)品分類 |
單克隆抗體 | 研究領(lǐng)域 |
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產(chǎn)品概述 |
Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.
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形式 |
This gene encodes a protein which is targeted to themedial Golgi apparatus and is necessary for posttranslationalmodification of dystroglycan. Mutations in this gene have beenassociated with congenital muscular dystrophy, mental retardation,and cerebellar cysts. Several alternatively spliced transcriptvariants of this gene have been described, but the full-lengthnature of some of these variants has not been determined. [providedby RefSeq].
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濃度 |
This FKRP antibody is generated from mice immunized with a KLH conjugated synthetic peptide between 418-444 amino acids from the C-terminal region of human FKRP. |
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純度 |
Mouse |
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試劑準(zhǔn)備 |
Purified monoclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein G column, followed by dialysis against PBS.
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復(fù)溶 |
Kawahara, G., et al. Hum. Mol. Genet. 19(4):623-633(2010)
Crowther-Swanepoel, D., et al. Nat. Genet. 42(2):132-136(2010)
Lu, P.J., et al. Biochim. Biophys. Acta 1802(2):253-258(2010)
Hanisch, F., et al. J. Neurol. 257(2):300-301(2010)
Bourteel, H., et al. J. Neurol. Neurosurg. Psychiatr. 80(12):1405-1408(2009)
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儲(chǔ)存條件 |
Human
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儲(chǔ)存溶液 |
Fukutin-related protein, 2---, FKRP
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應(yīng)用 |
WB~~1:100
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產(chǎn)品描述 |
Function
Transferase involved in the biosynthesis of the phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine- beta-3-N-acetylglucosamine-beta-4-(phosphate-6-)mannose), a carbohydrate structure present in alpha-dystroglycan (DAG1), which is required for binding laminin G-like domain-containing extracellular proteins with high affinity.
Cellular_Location
Golgi apparatus membrane; Single-pass type II membrane protein. Secreted. Cell membrane, sarcolemma. Rough endoplasmic reticulum. Note=According to some studies the N- terminal hydrophobic domain is cleaved after translocation to the Golgi apparatus and the protein is secreted. Localization at the cell membrane may require the presence of dystroglycan. At the Golgi apparatus localizes to the middle-to-trans-cisternae, as assessed by MG160 colocalization. Detected in rough endoplasmic reticulum in myocytes. In general, mutants associated with severe clinical phenotypes are retained within the endoplasmic reticulum
Tissue_Location
Expressed predominantly in skeletal muscle, placenta, and heart and relatively weakly in brain, lung, liver kidney and pancreas
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數(shù)量 |
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| 選擇 | 品牌 | 貨號(hào) | 產(chǎn)品名稱 | 規(guī)格 | 分類 | 研究領(lǐng)域 | 說(shuō)明書(shū) | 數(shù)量 | 目錄價(jià) | |
| 1 | Swant | 2D7 | monoclonal anti-Glutamate antibody | 200 μl | 單克隆抗體 | Antibodies against neurotransmitters |
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￥5096.00 | 訂購(gòu) 詢價(jià) | |
| 2 | Leading Biology | LM21248 | Anti-CD36 Antibody (Rabbit Monoclonal antibody) | 100 ul | 單克隆抗體 | Chondrogenesis Markers |
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| 3 | Swant | 288 | Monoclonal antibody to endothelial cells. | 單克隆抗體 |
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| 4 | Swant | 345 | Monoclonal antibody to rat pyramidal cells. | 200 μl | 單克隆抗體 | Miscellaneous antibodies |
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￥3243.00 | 訂購(gòu) 詢價(jià) | |
| 5 | Swant | 77 | mouse monoclonal antibody against rat angiotensinogen | 200 μl | 單克隆抗體 | Antibodies against components of the renin-angiotensin system |
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￥4015.00 | 訂購(gòu) 詢價(jià) | |
| 6 | Swant | 3A12 | monoclonal anti-GABA antibody | 200 μl | 單克隆抗體 | Antibodies against neurotransmitters |
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￥5096.00 | 訂購(gòu) 詢價(jià) |
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