抗体来源 Rabbit
克隆类型 polyclonal
交叉反应 Human, Mouse, Rat
产品类型 一抗
研究领域 细胞生物 神经生物学 转录调节因子 表观遗传学
蛋白分子量 predicted molecular weight: 81kDa
性 状 Lyophilized or Liquid
免 疫 原 KLH conjugated synthetic peptide derived from human BBS10
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
产品应用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500
(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.
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.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
巴尔得-别德尔综合征相关蛋白10抗体产品介绍 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.
Database links : UniProtKB/Swiss-Prot: Q8TAM1.2
BBS蛋白是一类研究早期儿童肥胖综合症有关的其中一种。巴尔得-别德尔综合征(Bardet-Biedl syndrome,BBS)的特征为不同程度的肥胖、智力延迟、色素视网膜病变、多指和肾脏异常。
纯度:在实验的任何阶段,确定抗体溶液纯度的最简单方法是取一部分样本进行SDS-PAGE电泳。凝胶可用考马斯亮蓝染色(灵敏度为0.1—0.5ug/带)或银染(灵敏度1~l0ug/带)。
定量:如果抗体还不纯,有一个快捷的定量方法,即通过SDS-PAGE电泳分离出轻、重链,然后和已知的标准染色带比较。如果需要分析许多样本,用免疫测定法对抗体定量较容易。如果抗体是经过纯化的,可通过测蛋白总量代替上述两种方法,有一简单的方法,即紫外吸收法。巴尔得-别德尔综合征相关蛋白10抗体的量可通过测280nm处的吸收值来测(10D大致相当于0.75mg/m1的纯化抗体)。
抗原结合活性:一般说来,纯化方法不会引起抗原结合活性的改变。用蛋白G或蛋白A树脂很少导致抗体活性丧失。然而,如果最终抗体产物的作用不如原来所预料的好,检测抗体纯化过程所丢失的活性就极为重要。用一系列滴定法比较纯化的抗体和其原材料的活性,以标定每一步中的总抗体量,这将有助于较好的估计通过纯化所丢失的活性。