FITC标记的二磷酸腺苷核糖基化因子6相互作用蛋白抗体-抗体-抗体-生物在线
上海沪震实业有限公司
FITC标记的二磷酸腺苷核糖基化因子6相互作用蛋白抗体

FITC标记的二磷酸腺苷核糖基化因子6相互作用蛋白抗体

商家询价

产品名称: FITC标记的二磷酸腺苷核糖基化因子6相互作用蛋白抗体

英文名称: Anti-ARL6/FITC

产品编号: HZ-6868R-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: IF=1:50-200

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 Rabbit Anti-ARL6/FITC Conjugated antibody

FITC标记的二磷酸腺苷核糖基化因子6相互作用蛋白抗体

 

英文名称 Anti-ARL6/FITC
中文名称 FITC标记的二磷酸腺苷核糖基化因子6相互作用蛋白抗体
别    名 ADP ribosylation factor like 6; ADP ribosylation factor like protein 6; ADP-ribosylation factor-like protein 6; Arl6; ARL6_HUMAN; Bardet Biedl syndrome 3 protein; Bardet-Biedl syndrome 3 protein; BBS3; MGC32934.  
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  信号转导  细胞凋亡  G蛋白信号  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Horse, 
产品应用 IF=1:50-200  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 20kDa
细胞定位 细胞膜 
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ARMER
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:
The protein encoded by this gene belongs to the ARF family of GTP-binding proteins. ARF proteins are important regulators of cellular traffic and are the founding members of an expanding family of homologous proteins and genomic sequences. They depart from other small GTP-binding proteins by a unique structural device that implements front-back communication from the N-terminus to the nucleotide-binding site. Studies of the mouse ortholog of this protein suggest an involvement in protein transport, membrane trafficking, or cell signaling during hematopoietic maturation. Alternative splicing occurs at this locus and two transcript variants encoding the same protein have been described. [provided by RefSeq, Jul 2008].

Function:
Involved in membrane protein trafficking at the base of the ciliary organelle. Mediates recruitment onto plasma membrane of the BBSome complex which would constitute a coat complex required for sorting of specific membrane proteins to the primary cilia. May regulate cilia assembly and disassembly and subsequent ciliary signaling events such as the Wnt signaling cascade. Isoform 2 may be required for proper retinal function and organization.

Subunit:
Interacts with SEC61B, ARL6IP1, ARL6IP2, ARL6IP3, ARL6IP4 ARL6IP5 and ARL6IP6. Interacts (GTP-bound form) with the BBSome a complex that contains BBS1, BBS2, BBS4, BBS5, BBS7, BBS8, BBS9 and BBIP10.

Subcellular Location:
Cell projection, cilium membrane; Peripheral membrane protein; Cytoplasmic side. Cytoplasm, cytoskeleton, cilium axoneme. Cytoplasm, cytoskeleton, cilium basal body. Note=Appears in a pattern of punctae flanking the microtubule axoneme that likely correspond to small membrane-associated patches. Localizes to the so-called ciliary gate where vesicles carrying ciliary cargo fuse with the membrane.

DISEASE:
Defects in ARL6 are a cause of Bardet-Biedl syndrome type 3 (BBS3) [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.
Defects in ARL6 are the cause of retinitis pigmentosa type 55 (RP55) [MIM:613575]. RP55 is a retinal dystrophy belonging to the group of pigmentary retinopathies. Retinitis pigmentosa is characterized by retinal pigment deposits visible on fundus examination and primary loss of rod photoreceptor cells followed by secondary loss of cone photoreceptors. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well.

Similarity:
Belongs to the small GTPase superfamily. Arf family.

Database links:

Entrez Gene: 84100 Human

Omim: 608845 Human

SwissProt: Q9H0F7 Human

Unigene: 373801 Human



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

该基因编码的蛋白属于GTP结合蛋白的ARF家族。ARF蛋白是细胞流量的重要调节因子,是一个不断扩展的同源蛋白家族和基因组序列的创始成员。它们通过一种独特的结构装置脱离其它小GTP结合蛋白,实现从N-末端到核苷酸结合位点的前向通信。该蛋白的小鼠直系同源物的研究提示在造血成熟过程中参与蛋白质转运、膜运输或细胞信号转导。在该位点发生选择性剪接,并描述了编码相同蛋白质的两个转录变体。[ RefSeq,JUL 2008 ]提供。