FITC标记的巴尔得-别德尔综合征相关蛋白10抗体-抗体-抗体-生物在线
FITC标记的巴尔得-别德尔综合征相关蛋白10抗体

FITC标记的巴尔得-别德尔综合征相关蛋白10抗体

商家询价

产品名称： FITC标记的巴尔得-别德尔综合征相关蛋白10抗体

英文名称： Anti-BBS10/FITC

产品编号： HZ-11512R-FITC

产品价格： null

产品产地： 中国/上海

品牌商标： HZbscience

更新时间： 2023-08-17T10:24:20

使用范围： ICC=1:50-200 IF=1:50-200

上海沪震实业有限公司
  • 联系人 : 鲍丽雯
  • 地址 : 上海市闵行区闵北路88弄1-30号第22幢AQ136室
  • 邮编 : 200612
  • 所在区域 : 上海
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  • 邮箱 : www.shzbio.net
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 Rabbit Anti-BBS10/FITC Conjugated antibody

FITC标记的巴尔得-别德尔综合征相关蛋白10抗体

 

产品编号 bs-11512R-FITC
英文名称 Anti-BBS10/FITC
中文名称 FITC标记的巴尔得-别德尔综合征相关蛋白10抗体
别    名 Bardet Biedl syndrome 10 protein; Bardet Biedl syndrome 10 protein homolog; C12orf58; FLJ23560; RGD1560748; BBS10_HUMAN.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  神经生物学  转录调节因子  表观遗传学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, 
产品应用 ICC=1:50-200 IF=1:50-200  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 81kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
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 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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.
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 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

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

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
   

BARDET Biedl综合征（Baldt Biedl综合征）是一种多效性遗传性疾病，其特征是肥胖、感光细胞退化、多指畸形、肾功能减退、肾功能异常和发育迟缓。BBS患者的其他相关临床表现包括糖尿病、高血压和先天性心脏缺陷。BBS基因定位于多个位点，编码14个蛋白BSBS1-BBS14。许多BBS基因编码基体或纤毛蛋白，提示BBS是睫状体功能障碍。BBS10（BARDET BieDL综合征），又称为第十二号染色体开放阅读名号58，C12ORF58或FLJ23 560，是一种新的723氨基酸蛋白，属于TCP-1伴侣蛋白家族。BBS10定位于原纤毛的基部，并在ATP水解时有助于蛋白质折叠。BBS10的抑制已发现损害纤毛发生，激活糖原合成酶激酶3途径，并导致过氧化物酶体增殖物激活受体核积累。编码BBS10的基因含有两个外显子，并映射到人类染色体12q21.2。