| 产品编号 | bs-11505R |
| 英文名称 | BBS12 |
| 中文名称 | 巴尔得-别德尔综合征相关蛋白12抗体 |
| 别 名 | Bardet Biedl syndrome 12 protein; Bardet-Biedl syndrome 12 (human); Bardet-Biedl syndrome 12 protein homolog; BBS12 gene; C4orf24; FLJ35630; FLJ41559; Gm1805; Gm407; Gm721; RP23-137F6.2;BBS12_HUMAN. |
| 研究领域 | 肿瘤 细胞生物 神经生物学 内分泌病 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | (predicted: Human,Mouse,Cow,Horse,Rabbit) |
| 产品应用 | WB=1:500-2000, ELISA=1:5000-10000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理论分子量 | 79kDa |
| 细胞定位 | 细胞浆 |
| 性 状 | Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human BBS12: 25-100/710 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 缓 冲 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 产品介绍 |
The protein encoded by this gene is part of a complex that is involved in membrane trafficking. The encoded protein is a molecular chaperone that aids in protein folding upon ATP hydrolysis. This protein also plays a role in adipocyte differentiation. Defects in this gene are a cause of Bardet-Biedl syndrome type 12. Two transcript variants encoding the same protein have been found for this gene. Function: Probable molecular chaperone. Assists the folding of proteins upon ATP hydrolysis. As part of the BBS/CCT complex may play a role in the assembly of BBSome, a complex involved in ciliogenesis regulating transports vesicles to the cilia. Involved in adipogenic differentiation. Subunit: Component of the BBS/CCT complex composed at least of MKKS, BBS10, BBS12, TCP1, CCT2, CCT3, CCT4, CCT5 AND CCT8. Subcellular Location: Cell projection, cilium. Note: Located within the basal body of the primary cilium of differentiating preadipocytes. DISEASE: Defects in BBS12 are the cause of Bardet-Biedl syndrome type 12 (BBS12) [MIM:209900]. Bardet-Biedl syndrome (BBS) is a genetically heterogeneous, autosomal recessive disorder characterized by usually severe pigmentary retinopathy, early onset obesity, polydactyly, hypogenitalism, renal malformation and mental retardation. Secondary features include diabetes mellitus, hypertension and congenital heart disease. A relatively high incidence of BBS is found in the mixed Arab populations of Kuwait and in Bedouin tribes throughout the Middle East, most likely due to the high rate of consaguinity in these populations and a founder effect. BBS12 seems to be rarely involved in oligogenic inheritance. Similarity: Belongs to the TCP-1 chaperonin family. BBS12 subfamily. SWISS: Q6ZW61 Gene ID: 166379 Database links: Entrez Gene: 166379 Human Omim: 610683 Human SwissProt: Q6ZW61 Human Unigene: 400698 Human |
| 1、抗体溶解方法 | |
| 2、抗体修复方式 | |
| 3、常用试剂的配制 | |
| 4、免疫组化操作步骤 | |
| 5、免疫组化问题解答 | |
| 6、Western Blotting 操作步骤 | |
| 7、Western Blotting 问题解答 | |
| 8、关于肽链的设计 | |
| 9、多肽的溶解与保存 | |
| 10、酶标抗体效价测定程序 | |
