anti-BBS4 antibody
CAT.NO. : ARG55131
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概述
产品描述 | Mouse Monoclonal antibody recognizes BBS4 |
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反应物种 | Hu, Ms, Rat |
应用 | WB |
宿主 | Mouse |
克隆 | Monoclonal |
克隆号 | 1292CT845.130.218 |
同位型 | IgG1 |
靶点名称 | BBS4 |
抗原物种 | Human |
抗原 | Recombinant protein corresponding to aa. 1-240 of Human BBS4. |
偶联标记 | Un-conjugated |
別名 | Bardet-Biedl syndrome 4 protein |
应用说明
应用建议 |
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应用说明 | * The dilutions indicate recommended starting dilutions and the optimal dilutions or concentrations should be determined by the scientist. | ||||
阳性对照 | HeLa |
属性
形式 | Liquid |
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纯化 | Purification with Protein G. |
缓冲液 | PBS and 0.09% (W/V) Sodium azide |
抗菌剂 | 0.09% (W/V) Sodium azide |
存放说明 | For continuous use, store undiluted antibody at 2-8°C for up to a week. For long-term storage, aliquot and store at -20°C or below. Storage in frost free freezers is not recommended. Avoid repeated freeze/thaw cycles. Suggest spin the vial prior to opening. The antibody solution should be gently mixed before use. |
注意事项 | For laboratory research only, not for drug, diagnostic or other use. |
生物信息
数据库连接 | Swiss-port # Q8C1Z7 Mouse Bardet-Biedl syndrome 4 protein homolog |
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基因名称 | BBS4 |
全名 | Bardet-Biedl syndrome 4 |
背景介绍 | This gene is a member of the Bardet-Biedl syndrome (BBS) gene family. Bardet-Biedl syndrome is an autosomal recessive disorder characterized by severe pigmentary retinopathy, obesity, polydactyly, renal malformation and mental retardation. The proteins encoded by BBS gene family members are structurally diverse. The similar phenotypes exhibited by mutations in BBS gene family members are likely due to the protein's shared roles in cilia formation and function. Many BBS proteins localize to the basal bodies, ciliary axonemes, and pericentriolar regions of cells. BBS proteins may also be involved in intracellular trafficking via microtubule-related transport. The protein encoded by this gene has sequence similarity to O-linked N-acetylglucosamine (O-GlcNAc) transferases in plants and archaebacteria and in human forms a multi-protein "BBSome" complex with seven other BBS proteins. Alternative splice variants have been described but their predicted protein products have not been experimentally verified.[provided by RefSeq, Oct 2014] |
生物功能 | The BBSome complex is thought to function as a coat complex required for sorting of specific membrane proteins to the primary cilia. The BBSome complex is required for ciliogenesis but is dispensable for centriolar satellite function. This ciliogenic function is mediated in part by the Rab8 GDP/GTP exchange factor, which localizes to the basal body and contacts the BBSome. Rab8(GTP) enters the primary cilium and promotes extension of the ciliary membrane. Firstly the BBSome associates with the ciliary membrane and binds to RAB3IP/Rabin8, the guanosyl exchange factor (GEF) for Rab8 and then the Rab8-GTP localizes to the cilium and promotes docking and fusion of carrier vesicles to the base of the ciliary membrane. The BBSome complex, together with the LTZL1, controls SMO ciliary trafficking and contributes to the sonic hedgehog (SHH) pathway regulation. Required for proper BBSome complex assembly and its ciliary localization. Required for microtubule anchoring at the centrosome but not for microtubule nucleation. May be required for the dynein-mediated transport of pericentriolar proteins to the centrosome. [UniProt] |
细胞定位 | Cytoplasm, cytoskeleton, microtubule organizing center, centrosome. Cytoplasm, cytoskeleton. Cell projection, cilium membrane. Cytoplasm. Cytoplasm, cytoskeleton, microtubule organizing center, centrosome, centriolar satellite Cell projection, cilium, flagellum. Note=Localizes to the pericentriolar region throughout the cell cycle Centrosomal localization requires dynein |
研究领域 | Neuroscience antibody |
预测分子量 | 58 kDa |
