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货号： bs-4414R-AF594 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-4414R-AF594
- 英文名称
- Anti-Nyctalopin/AF594
- 中文名称
- AF594标记的夜盲蛋白NYX抗体
- 别 名
- CLRP; CSNB1; CSNB4; leucine-rich repeat protein; Nyctalopin; NYX; NYX_HUMAN.
- 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 干细胞
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat,
- 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
- 50kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 2mg/1ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human Nyctalopin
- 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 product of this gene belongs to the small leucine-rich proteoglycan (SLRP) family of proteins. Defects in this gene are the cause of congenital stationary night blindness type 1 (CSNB1), also called X-linked congenital stationary night blindness (XLCSNB). CSNB1 is a rare inherited retinal disorder characterized by impaired scotopic vision, myopia, hyperopia, nystagmus and reduced visual acuity. The role of other SLRP proteins suggests that mutations in this gene disrupt developing retinal interconnections involving the ON-bipolar cells, leading to the visual losses seen in patients with complete CSNB. [provided by RefSeq, Oct 2008]
Subcellular Location:
Secreted > extracellular space > extracellular matrix.
Tissue Specificity:
Expressed in kidney and retina. Also at low levels in brain, testis and muscle. Within the retina, expressed in the inner segment of photoreceptors, outer and inner nuclear layers and the ganglion cell layer.
DISEASE:
Defects in NYX are the cause of congenital stationary night blindness type 1A (CSNB1A) [MIM:310500]; also called X-linked congenital stationary night blindness (XLCSNB). Congenital stationary night blindness is a non-progressive retinal disorder characterized by impaired night vision. CSNB1A is characterized by impaired scotopic vision, myopia, hyperopia, nystagmus and reduced visual acuity.
Similarity:
Belongs to the small leucine-rich proteoglycan (SLRP) family.
SLRP class IV subfamily.
Contains 11 LRR (leucine-rich) repeats.
Contains 1 LRRCT domain.
Contains 1 LRRNT domain.
Database links:Entrez Gene: 60506Human
Omim: 300278Human
SwissProt: Q9GZU5Human
Unigene: 302019Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.