FITC标记的视网膜色素变性蛋白1抗体
产品名称： FITC标记的视网膜色素变性蛋白1抗体
英文名称： Anti-RP1/FITC
产品编号： HZ-11030R-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： ICC=1:50-200 IF=1:50-200
上海沪震实业有限公司
- 联系人 : 鲍丽雯
- 地址 : 上海市闵行区闵北路88弄1-30号第22幢AQ136室
- 邮编 : 200612
- 所在区域 : 上海
- 电话 : 139****0749 点击查看
- 传真 : 点击查看
- 邮箱 : www.shzbio.net
- 二维码 : 点击查看
Rabbit Anti-RP1/FITC Conjugated antibody
FITC标记的视网膜色素变性蛋白1抗体
| 产品编号 | bs-11030R-FITC |
| 英文名称 | Anti-RP1/FITC |
| 中文名称 | FITC标记的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. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 发育生物学 神经生物学 细胞粘附分子 细胞骨架 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Horse, Rabbit, |
| 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 | 241kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 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 | 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: 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. Similarity: Contains 2 doublecortin domains. Database links: Entrez Gene: 6101 Human Omim: 603937 Human SwissProt: P56715 Human Unigene: 128938 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
视网膜色素变性1是一种新的2156氨基酸氧调节的视网膜感光体。最初命名为Orp1（对于‘氧调节蛋白-1’），视网膜色素变性1的表达已被视网膜中的氧水平调节。视网膜色素变性1基因突变引起视网膜色素变性占主导地位，导致视网膜感光细胞变性和症状，如夜视失明和中外视野视野缺损。视网膜色素变性5月1日协助光感受器细胞的分化，并已在光感受器的纤毛中识别，可能有助于光感受器的内部和外部段的纤毛结构和蛋白质转运。视网膜色素变性1包含两个双胚乳结构域，并由一个映射到人类染色体8q11-q13的基因编码。