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Rabbit Anti-ZSWIM3/BF350 Conjugated antibody (bs-9138R-BF350)
~~~促销,代码KXJ230206-6~~~
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-9138R-BF350
英文名称 Anti-ZSWIM3/BF350
中文名称 BF350标记的ZSWIM3蛋白抗体
别    名 Zinc finger SWIM domain containing protein 3; C20orf164; Zinc finger SWIM type containing 3; ZSWIM 3; ZSWM3_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  免疫学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
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 79kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ZSWIM3
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:
Zinc-finger proteins contain DNA-binding domains and have a wide variety of functions, most of which encompass some form of transcriptional activation or repression. ZSWIM3 (Zinc finger SWIM domain-containing protein 3) is a 696 amino acid protein that contains one SWIM-type zinc finger. SWIM domains are found in a variety of eukaryotic and prokaryotic proteins and are thought to be critical for certain ubiquitination reactions. The gene encoding ZSWIM3 maps to human chromosome 20, which contains nearly 63 million bases that encode over 600 genes, some of which are associated with Creutzfeldt-Jakob disease, amyotrophic lateral sclerosis, spinal muscular atrophy, ring chromosome 20 epilepsy syndrome and Alagille syndrome.

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.

Subunit:
Part of BBSome complex, that contains BBS1, BBS2, BBS4, BBS5, BBS7, BBS8, BBS9 and BBIP10. The BBSome complex binds to PCM1 and tubulin. Binds to phosphoinositides.

Subcellular Location:
Cell projection, cilium membrane. Cytoplasm. Cytoplasm, cytoskeleton, cilium basal body.

DISEASE:
Defects in BBS5 are a cause of Bardet-Biedl syndrome type 5 (BBS5) [MIM:209900]. Bardet-Biedl syndrome (BBS) is a genetically heterogeneous disorder characterized by usually severe pigmentary retinopathy, early onset obesity, polydactyly, hypogenitalism, renal malformation and mental retardation. Secondary features include diabetes mellitus, hypertension and congenital heart disease. A relatively high incidence of BBS is found in the mixed Arab populations of Kuwait and in Bedouin tribes throughout the Middle East, most likely due to the high rate of consaguinity in these populations and a founder effect.

Similarity:
Belongs to the BBS5 family.

Database links:
UniProtKB/Swiss-Prot: Q96MP5.2

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