货号 |
bs-11510R-1 |
品牌 |
|
浓度 |
|
货期 |
现货 |
英文名称 |
Rabbit Anti-TTC8 antibody |
中文名称 |
Rabbit Anti-TTC8 antibody |
研究领域 |
肿瘤,细胞生物,神经生物学,内分泌病, |
英文别名 |
Bardet Biedl syndrome 8 protein; Bardet Biedl syndrome type 8; Bardet-Biedl syndrome 8 protein; BBS8; Tetratricopeptide repeat domain 8; Tetratricopeptide repeat protein 8; TPR repeat protein 8; TTC 8; Ttc8; TTC8_HUMAN. |
反应物种(已验证) |
Human,Mouse |
反应物种(预测) |
Rat,Chicken,Dog,Pig,Cow,Horse,Rabbit,Sheep |
产品应用(已验证) |
WB,IHC,FCM |
产品应用(可推荐) |
ICC,IF,ELISA |
推荐稀释比例 |
WB=1:500-2000,Elisa=1:5000-10000,IHC-P=1:100-500,IHC-F=1:100-500,Flow Cyt=0.2ug/test,IF=1:100-500,ICC=1:100-500, |
克隆类型 |
多克隆 |
抗体来源 |
Rabbit |
理论分子量 |
61 |
细胞定位 |
细胞浆,细胞膜 |
性状 |
Liquid |
免疫原 |
KLH conjugated synthetic peptide derived from human BBS8 |
抗原表位 |
251-330/541 |
亚型 |
IgG |
纯化方法 |
affinity purified by Protein A |
SUBCELLULAR |
Cytoplasm, cytoskeleton, centrosome. Cell projection, cilium membrane. Cytoplasm. Note=Localizes to nonmembranous centriolar satellites in the cytoplasm. |
Tissue |
Widely expressed. |
SIMILARITY |
Contains 8 TPR repeats. |
SUBUNIT |
Part of BBSome complex, that contains BBS1, BBS2, BBS4, BBS5, BBS7, BBS8, BBS9 and BBIP10. The BBSome complex binds to PCM1 and tubulin. Interacts with PCM1. Interacts with CCDC28B. |
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) en |
DISEASE |
Defects in TTC8 are the cause of retinitis pigmentosa type 51 (RP51) [MIM:613464]. It is a retinal dystrophy belonging to the group of pigmentary retinopathies. Retinitis pigmentosa is characterized by retinal pigment deposits visible on fundus examinatio |
SWISS |
Q8TAM2 |
Gene ID |
123016 |
保存条件 |
Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
Important Note |
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
英文介绍 |
Bardet-Biedl syndrome (BBS) is a pleiotropic genetic disorder characterized by obesity, photoreceptor degeneration, polydactyly, hypogenitalism, renal abnormalities, and developmental delay. BBS patients also have an increased risk of developing diabetes, hypertension, and congenital heart defects. BBS is a heterogeneous disorder mapping to eight genetic loci and encoding eight proteins, BBS1-BBS8. Five BBS proteins encode basal body or cilia proteins, suggesting that BBS is a ciliary dysfunction disorder. BBS2 contains two overlapping genes: BBS2L1 and BBS2L2. BBSL1 was re-named BBS7, whereas BBS2L2 independently funcitons as BBS1. BBS7 contains 672 amino acids and is expressed at low to moderate levels in most human tissues. |