X连锁先天性视网膜劈裂XLRS1蛋白抗体
规格：1mg/1ml
英文名： RS1
别名： Retinoschisin; RS1; X-linked juvenile retinoschisis protein; XLRS1_HUMAN.
分子量： 23kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human RS1
交叉反应：Human, Mouse, Rat, Chicken, Dog, Cow, Horse, Sheep,
细胞定位：分泌型蛋白
X连锁先天性视网膜劈裂XLRS1蛋白抗体产品介绍：background: This gene encodes an extracellular protein that plays a crucial role in the cellular organization of the retina. The encoded protein is assembled and secreted from photoreceptors and bipolar cells as a homo-oligomeric protein complex. Mutations in this gene are responsible for X-linked retinoschisis, a common, early-onset macular degeneration in males that results in a splitting of the inner layers of the retina and severe loss in vision. [provided by RefSeq, Oct 2008] Function: May be active in cell adhesion processes during retinal development. Subcellular Location: Secreted. Tissue Specificity: Restricted to the retina (at protein level). At the mRNA level, detected only within the photoreceptor cell layer, most prominently within the inner segments of the photoreceptors. Undetectable in the inner plexiform layers and the inner nuclear layer. At the protein level, found in the inner segment of the photoreceptors, the inner nuclear layer, the inner plexiform layer and the ganglion cell layer. At the macula, expressed in both the outer and inner nuclear layers and in the inner plexiform X连锁先天性视网膜劈裂XLRS1蛋白抗体layer (at protein level). DISEASE: Defects in RS1 are the cause of retinoschisis juvenile X-linked type 1 (XLRS1) [MIM:312700]. A vitreo-retinal dystrophy characterized by macular pathology and by splitting of the superficial layer of the retina. Macular changes are present in almost all cases. In the fundi, radially oriented intraretinal foveomacular cysts are seen in a spoke-wheel configuration, with the absence of foveal reflex in most cases. In addition, approximately half of cases have bilateral peripheral retinoschisis in the inferotemporal part of the retina. Aside from the typical fundus appearance, strabismus, nystagmus, axial hyperopia, defective color vision and foveal ectopy can X连锁先天性视网膜劈裂XLRS1蛋白抗体be present. The most important complications are vitreous hemorrhage, retinal detachment, and neovascular glaucoma. Similarity: Contains 1 F5/8 type C domain. Gene ID: 6247 Database links: Entrez Gene: 6247 Human Entrez Gene: 20147 Mouse Omim: 300839 Human SwissProt: O15537 Human SwissProt: Q9Z1L4 Mouse Unigene: 715725 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
X连锁先天性视网膜劈裂XLRS1蛋白抗体产品应用：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：**学 神经生物学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid