视锥视杆相关蛋白颁翱搁顿2抗体
规格：1尘驳/1尘濒
英文名： CORD2/OTX3
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.
分子量： 32kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human CORD2
交叉反应：Human, Mouse, Rat, Dog, Cow, Horse, Sheep, Cat,
细胞定位：细胞核
视锥视杆相关蛋白颁翱搁顿2抗体产物介绍：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. 视锥视杆相关蛋白颁翱搁顿2抗体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 视锥视杆相关蛋白颁翱搁顿2抗体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. Gene ID: 1406 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.
视锥视杆相关蛋白颁翱搁顿2抗体产物应用：WB=1:100-500 ELISA=1:500-1000 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.
研究领域：神经生物学 转录调节因子 表观遗传学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid