产品名称：Anti-ARL6/FITC抗体

Anti-ARL6/FITC抗体

产品编号YB-6868R-FITC

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.

Anti-ARL6/FITC抗体

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Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

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.

Anti-ARL6/FITC抗体

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.Anti-ARL6/FITC抗体 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:

Anti-ARL6/FITC抗体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.