FITC标记的视锥视杆相关蛋白CORD2抗体
产品名称： FITC标记的视锥视杆相关蛋白CORD2抗体
英文名称： Anti-CORD2/OTX3/FITC
产品编号： HZ-13990R-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
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Rabbit Anti-CORD2/OTX3/FITC Conjugated antibody
FITC标记的视锥视杆相关蛋白CORD2抗体
| 英文名称 | Anti-CORD2/OTX3/FITC |
| 中文名称 | FITC标记的视锥视杆相关蛋白CORD2抗体 |
| 别 名 | Cone rod homeobox; Cone rod homeobox protein; Cone-rod homeobox protein; CORD 2; CRD; CRX; CRX_HUMAN; LCA 7; LCA7; Orthodenticle homeobox 3; OTX 3; OTX3. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 神经生物学 转录调节因子 表观遗传学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Cow, Horse, Sheep, Cat, |
| 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 | 32kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human CORD2 |
| 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 is a photoreceptor-specific transcription factor which plays a role in the differentiation of photoreceptor cells. This homeodomain protein is necessary for the maintenance of normal cone and rod function. Mutations in this gene are associated with photoreceptor degeneration, Leber congenital amaurosis type III and the autosomal dominant cone-rod dystrophy 2. Several alternatively spliced transcript variants of this gene have been described, but the full-length nature of some variants has not been determined. [provided by RefSeq, Jul 2008] Function: Binds and transactivates the sequence 5'-TAATC[CA]-3' which is found upstream of several photoreceptor-specific genes, including the opsin genes. Acts synergistically with other transcription factors, e.g. NRL and RX, to regulate photoreceptor cell-specific gene transcription. Essential for the maintenance of mammalian photoreceptors. Subcellular Location: Nucleus. Tissue Specificity: Retina. DISEASE: Defects in CRX are the cause of Leber congenital amaurosis type 7 (LCA7) [MIM:613829]. LCA designates a clinically and genetically heterogeneous group of childhood retinal degenerations, generally inherited in an autosomal recessive manner. Affected infants have little or no retinal photoreceptor function as tested by electroretinography. LCA represents the most common genetic cause of congenital visual impairment in infants and children. Defects in CRX are the cause of cone-rod dystrophy type 2 (CORD2) [MIM:120970]; also known as cone-rod retinal dystrophy 2 (CRD2). CORDs are inherited retinal dystrophies belonging to the group of pigmentary retinopathies. CORDs are characterized by retinal pigment deposits visible on fundus examination, predominantly in the macular region, and initial loss of cone photoreceptors followed by rod degeneration. This leads to decreased visual acuity and sensitivity in the central visual field, followed by loss of peripheral vision. Severe loss of vision occurs earlier than in retinitis pigmentosa. Defects in CRX are a cause of retinitis pigmentosa (RP) [MIM:268000]. 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: Belongs to the paired homeobox family. Contains 1 homeobox DNA-binding domain. Database links: Entrez Gene: 1406 Human Entrez Gene: 280756 Cow Entrez Gene: 12951 Mouse Entrez Gene: 60446 Rat Omim: 602225 Human SwissProt: Q9XSK0 Cow SwissProt: Q8SQ03 Dog SwissProt: O43186 Human SwissProt: O54751 Mouse Unigene: 617342 Human Unigene: 633434 Human Unigene: 639114 Human Unigene: 441911 Mouse Unigene: 44287 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
该基因编码的蛋白质是光感受器特异性转录因子，在光感受器细胞的分化中起作用。这种同源结构域蛋白是维持正常圆锥和杆功能所必需的。该基因的突变与光感受器变性、Leber先天性黑蒙III型和常染色体显性锥杆营养不良有关。已经描述了该基因的几个选择性剪接的转录体变体，但一些变体的全长性质尚未确定。[ RefSeq，JUL 2008 ]