| 產(chǎn)品編號 | bs-11512R |
| 英文名稱 | Rabbit Anti-BBS10 antibody |
| 中文名稱 | 巴爾得-別德爾綜合征相關(guān)蛋白10抗體 |
| 別 名 | Bardet Biedl syndrome 10 protein; Bardet Biedl syndrome 10 protein homolog; C12orf58; FLJ23560; RGD1560748; BBS10_HUMAN. |
| 研究領(lǐng)域 | 細胞生物 神經(jīng)生物學 轉(zhuǎn)錄調(diào)節(jié)因子 表觀遺傳學 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human,Mouse,Rat) |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 81kDa |
| 細胞定位 | 細胞漿 細胞膜 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human BBS10: 51-130/723 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
Bardet-Biedl syndrome (BBS) is a pleiotropic genetic disorder characterized by obesity, photoreceptor degeneration, polydactyly, hypogenitalism, renal abnormalities, and developmental delay. Other associated clinical findings in BBS patients include diabetes, hypertension, and congenital heart defects. BBS genes map to multiple loci and encode fourteen proteins, BBS1-BBS14. Many BBS genes encode basal body or cilia proteins, suggesting that BBS is a ciliary dysfunction disorder. BBS10 (Bardet-Biedl syndrome 10), also known as chromosome 12 open reading fame 58, C12orf58 or FLJ23560, is a novel 723 amino acid protein belonging to the TCP-1 chaperonin family. BBS10 localizes to the basal body of primary cilium and assists in protein folding upon ATP hydrolysis. Inhibition of BBS10 has been found to impair ciliogenesis, activate the glycogen synthase kinase 3 pathway and cause peroxisome proliferator-activated receptor nuclear accumulation. The gene encoding BBS10 contains two exons and maps to human chromosome 12q21.2. Function: BBS10 belongs to the TCP-1 chaperonin family. It is a probable molecular chaperone; assist the folding of proteins upon ATP hydrolysis. Defects in BBS10 are the cause of Bardet-Biedl syndrome type 10 (BBS10) [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. 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 BBS10 are the cause of Bardet-Biedl syndrome type 10 (BBS10) [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. Similarity: Belongs to the TCP-1 chaperonin family. SWISS: Q8TAM1 Gene ID: 79738 Database links: Entrez Gene: 79738 Human Omim: 610148 Human SwissProt: Q8TAM1 Human Unigene: 96322 Human BBS蛋白是一類研究早期兒童肥胖綜合癥有關(guān)的其中一種。巴爾得-別德爾綜合征（Bardet-Biedl syndrome，BBS）的特征為不同程度的肥胖、智力延遲、色素視網(wǎng)膜病變、多指和腎臟異常。 |
| 1、抗體溶解方法 | |
| 2、抗體修復方式 | |
| 3、常用試劑的配制 | |
| 4、免疫組化操作步驟 | |
| 5、免疫組化問題解答 | |
| 6、Western Blotting 操作步驟 | |
| 7、Western Blotting 問題解答 | |
| 8、關(guān)于肽鏈的設(shè)計 | |
| 9、多肽的溶解與保存 | |
| 10、酶標抗體效價測定程序 | |
