产品编号 | bs-13990R-BF647 |
英文名称 | Rabbit Anti-CORD2/OTX3/BF647 Conjugated antibody |
中文名称 | BF647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 | Rat, (predicted: Human, Mouse, 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: 12951 Mouse Omim: 602225 Human 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. |
1、抗体溶解方法 | |
2、抗体修复方式 | |
3、常用试剂的配制 | |
4、免疫组化操作步骤 | |
5、免疫组化问题解答 | |
6、Western Blotting 操作步骤 | |
7、Western Blotting 问题解答 | |
8、关于肽链的设计 | |
9、多肽的溶解与保存 | |
10、酶标抗体效价测定程序 | |