簡要描述:巴爾得-別德爾綜合征相關(guān)蛋白10抗體免 疫 原;KLH conjugated synthetic peptide derived from human BBS10: 51-130/723
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巴爾得-別德爾綜合征相關(guān)蛋白10抗體
產(chǎn)品名稱 | 英文名稱 | 貨號 |
巴爾得-別德爾綜合征相關(guān)蛋白10抗體 | BBS10 | GOY-01K2286 |
商品屬性:
英文名稱: BBS10
中文名: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(xì)胞生物 神經(jīng)生物學(xué) 轉(zhuǎn)錄調(diào)節(jié)因子 表觀遺傳學(xué)
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 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (石蠟切片需做抗原修復(fù))
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量;81kDa
細(xì)胞定位;細(xì)胞漿 細(xì)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, 0.03% Proclin300 and 50% Glycerol.
注意事項;This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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. 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. |
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