全部产品分类
品牌 |
Leading Biology | 货号 |
LM20352 |
产品分类 |
单克隆抗体 | 研究领域 |
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产品概述 |
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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浓度 |
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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纯度 |
Mouse |
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试剂准备 |
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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复溶 |
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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储存条件 |
Human
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储存溶液 |
Fukutin-related protein, 2---, FKRP
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应用 |
WB~~1:100
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产品描述 |
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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数量 |
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| 选择 | 品牌 | 货号 | 产品名称 | 规格 | 分类 | 研究领域 | 说明书 | 数量 | 目录价 | |
| 1 | Swant | 2D7 | monoclonal anti-Glutamate antibody | 200 µl | 单克隆抗体 | Antibodies against neurotransmitters |
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￥5096.00 | 订购 询价 | |
| 2 | Leading Biology | LM21248 | Anti-CD36 Antibody (Rabbit Monoclonal antibody) | 100 ul | 单克隆抗体 | Chondrogenesis Markers |
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| 3 | Swant | 288 | Monoclonal antibody to endothelial cells. | 单克隆抗体 |
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￥3243.00 | 订购 询价 | |||
| 4 | Swant | 345 | Monoclonal antibody to rat pyramidal cells. | 200 µl | 单克隆抗体 | Miscellaneous antibodies |
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￥3243.00 | 订购 询价 | |
| 5 | Swant | 3A12 | monoclonal anti-GABA antibody | 200 µl | 单克隆抗体 | Antibodies against neurotransmitters |
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￥5096.00 | 订购 询价 | |
| 6 | Swant | 77 | mouse monoclonal antibody against rat angiotensinogen | 200 µl | 单克隆抗体 | Antibodies against components of the renin-angiotensin system |
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￥4015.00 | 订购 询价 |
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