巴德-毕德氏综合征蛋白BBS5抗体

英文名称    BBS5    

中文名称    巴德-毕德氏综合征蛋白BBS5抗体    

别    名    Bardet Biedl syndrome 5 protein; BBS 5; BBS-5.    

说 明 书    0.2ml      

研究领域    肿瘤  细胞生物  神经生物学      

抗体来源    Rabbit    

克隆类型    Polyclonal    

交叉反应    Human, Mouse, Rat, Dog, Pig, Cow, Horse,     

产品应用    WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200 巴德-毕德氏综合征蛋白BBS5抗体（石蜡切片需做抗原修复） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.    

分 子 量    38kDa    

细胞定位    细胞浆 细胞膜     

性    状    Lyophilized or Liquid    

浓    度    1mg/1ml    

免 疫 原    KLH conjugated synthetic peptide derived from human BBS5    

亚    型    IgG    

纯化方法    affinity purified by Protein A    

储 存 液    Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4    

保存条件    Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.    

巴德-毕德氏综合征蛋白BBS5抗体产品介绍    background:

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 is a heterogeneous disorder; BBS genes map to eight genetic loci and encode eight proteins, BBS1-BBS8. Five BBS genes encode basal body or cilia proteins, suggesting that BBS is a ciliary dysfunction disorder. BBS5 localizes to ciliary basal bodies and is a member of the basal body/flagellar proteome. It plays a role in flagellar and basal body assembly and function. A mutation or loss of BBS5 may be correlated with photoreceptor degeneration

Function:
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.    

巴德-毕德氏综合征蛋白BBS5抗体

hz-1156 GM-CSF(Human Granulocyte-Macrophage Colony Stimulating Factor) ELISA Kit  人粒细胞巨噬细胞集落刺激因子

hz-1157 GDNF(Human glial cell line-derived neurotrophic factor) ELISA KIT  人胶质细胞系来源的神经营养因子

hz-1157 PA-IgG/M/A ELISA Kit  大鼠抗血小板抗体IgG/M/A

hz-1157 RANTES/CCL5(Mouse regulated on activation in normal T-cell expressed and secreted) ELISA Kit  小鼠正常T细胞表达和分泌因子

hz-1158 MIP-2(Mouse Macrophage Inflammatory Protein 2) ELISA Kit  小鼠巨噬细胞炎性蛋白2

hz-1158 NTX)ELISA Kit  大鼠Ⅰ型胶原N末端肽

hz-1158 G-CSF(Human Granulocyte Colony Stimulating Factor) ELISA Kit  人粒细胞集落刺激因子

hz-1159 NAP-2/CXCL7(Human neutrophil activating protein-2) ELISA Kit  人中性粒细胞趋化蛋白2

hz-1159 HSP-27 ELISA Kit  大鼠热休克蛋白27

hz-1159 OT(Mouse oxytocin) ELISA Kit  小鼠催产素

hz-1160 MIP-3 Beta/ELC/CCL19(Mouse macrophage inflammatory protein 3 Beta) ELISA Kit  小鼠巨噬细胞炎性蛋白3β

hz-1160 大鼠Smad1 ELISA Kit   大鼠Smad1

hz-1160 Human fractalkine/CX3CL1 ELISA Kit   人趋化因子

hz-1161 大鼠Smad7 ELISA Kit   大鼠Smad7

hz-1161 NTX(Mouse cross linked N-telopeptide of type I collagen) ELISA Kit  小鼠Ⅰ型胶原N末端肽

hz-1161 bFGF-9(Human basic fibroblast growth factor 9) ELISA Kit  人碱性成纤维细胞生长因子9

hz-116 5-NT(Mouse 5-Nucleotidase) ELISA Kit  小鼠5核苷酸酶

hz-1162 26S PSM ELISA Kit   大鼠26S蛋白酶体

hz-1162 bFGF-6(Human basic fibroblast growth factor 6) ELISA Kit  人碱性成纤维细胞生长因子6

hz-1163 bFGF-4(Human basic fibroblast growth factor 4) ELISA Kit  人碱性成纤维细胞生长因子4

hz-1163 TIEG1 ELISA Kit   大鼠TGF-β诱导早期基因1

hz-1163 CD19(Rat Troponin T) ELISA Kit  小鼠CD19分子

hz-1164 CD3(Rat Troponin T) ELISA Kit  小鼠CD3分子

hz-1164 visfatin ELISA Kit  大鼠内脂素/内脏脂肪素

hz-1164 aFGF-1(Human acidic fibroblast growth factor 1) ELISA Kit  人酸性成纤维细胞生长因子1

hz-1165 TAP ELISA Kit  大鼠胰蛋白酶原激活肽