X连锁先天性视网膜劈裂XLRS1蛋白抗体

英文名称    RS1    

中文名称    X连锁先天性视网膜劈裂XLRS1蛋白抗体    

别    名    Retinoschisin; RS1; X-linked juvenile retinoschisis protein; XLRS1_HUMAN.    

X连锁先天性视网膜劈裂XLRS1蛋白抗体         

说 明 书    0.2ml      

研究领域    免疫学  神经生物学      

抗体来源    Rabbit    

克隆类型    Polyclonal    

交叉反应    Human, Mouse, Rat, Chicken, Dog, Cow, Horse, Sheep,     

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.    

分 子 量    23kDa    

细胞定位    分泌型蛋白     

性    状    Lyophilized or Liquid    

浓    度    1mg/1ml    

免 疫 原    KLH conjugated synthetic peptide derived from human RS1    

亚    型    IgG    

纯化方法    affinity purified by Protein A    

储 存 液    Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4    

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.    

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 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 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 

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