网膜色素变性蛋白1抗体

我公司专业供应网膜色素变性蛋白1抗体。了解更多关于的网膜色素变性蛋白1抗体英文名称网膜色素变性蛋白1抗体产品别名规格请看以下说明书。

英文名称  Anti-RP1/DCDC4A

中文名称  视网膜色素变性蛋白1抗体

别    名  DCDC4A; ORP1; Oxygen-regulated protein 1; Retinitis pigmentosa 1 protein; Retinitis pigmentosa RP1 protein; RP1; RP1_HUMAN.

浓    度  1mg/1ml

规 格  0.2ml/200μg

网膜色素变性蛋白1抗体的功能：

抗体的主要功能是与抗原（包括外来的和自身的）相结合，从而有效地清除侵入机体内的微生物、寄生虫等异物，抗体（antibody）是一种应答抗原产生的、可与抗原特异性结合的蛋白质。每种抗体与特定的抗原决定基结合。这种结合可以使抗原失活，也可能无效但有时也会对机体造成病理性损害，如抗核抗体、抗双链DNA抗体、抗甲状腺球蛋白抗体等一些自身抗体的产生，对人体可造成危害。                                                                       

抗体来源  Rabbit  

克隆类型  polyclonal

交叉反应  Human, Mouse, Rat, Dog, Horse, Rabbit  

产品类型  一抗    

研究领域  发育生物学 神经生物学 细胞粘附分子 细胞骨架  

蛋白分子量  predicted molecular weight: 241kDa

性    状  Lyophilized or Liquid

免 疫 原  KLH conjugated synthetic peptide derived from human RP1/DCDC4A

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

产品应用   ELISA=1:500-1000  IP=1:20-100  IHC-P=1:100-500  IHC-F=1:100-500  ICC=1:100-500  IF=1:100-500

（石蜡切片需做抗原修复）

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

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.

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

产品介绍 Retinitis pigmentosa 1 is a novel 2,156 amino acid oxygen-regulated photoreceptor specific to retina. Originally named ORP1 (for 'oxygen-regulated protein-1'), the expression of retinitis pigmentosa 1 has been found to be regulated by oxygen levels in the retina. Mutation of the retinitis pigmentosa 1 gene causes dominant retinitis pigmentosa which leads to degeneration of retinal photoreceptor cells and symptoms such as night vision blindness and deficits in the midperipheral visual field. Retinitis pigmentosa 1 may assist in differentiation of photoreceptor cells and has been identified in the cilia of photoreceptors, possibly aiding in both cilial structure and protein transport between inner and outer segments of photoreceptors. Retinitis pigmentosa 1 contains two doublecortin domains and is encoded by a gene which maps to human chromosome 8q11-q13.

Function : Microtubule-associated protein regulating the stability and length of the microtubule-based axoneme of photoreceptors. Required for the differentiation of photoreceptor cells, it plays a role in the organization of the outer segment of rod and cone photoreceptors ensuring the correct orientation and higher order stacking of outer segment disks along the photoreceptor axoneme.

Subunit : Interacts (via the doublecortin domains) with microtubules. Interacts with RP1L1 (By similarity). Interacts with MAK (By similarity).

Subcellular Location : Cytoplasm; cytoskeleton; cilium axoneme. Cell projection; cilium; photoreceptor outer segment. Specifically localized in the connecting cilia of rod and cone photoreceptors.

Tissue Specificity : Expressed in retina. Not expressed in heart, brain, placenta, lung, liver, skeletal muscle, kidney, spleen and pancreas.

DISEASE : Defects in RP1 are the cause of retinitis pigmentosa type 1 (RP1) [MIM:180100]. RP leads to degeneration of retinal photoreceptor cells. 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.

网膜色素变性蛋白1抗体Similarity : Contains 2 doublecortin domains.

Database links : UniProtKB/Swiss-Prot: P56715.1