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货号： bs-9973R-Bio 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-9973R-Bio
- 英文名称
- Anti-LCA5/Lebercilin/Biotin
- 中文名称
- 生物素标记的致盲基因LCA5蛋白抗体
- 别 名
- C6orf152; LCA5; Leber congenital amaurosis 5; Leber congenital amaurosis 5 protein; ORF64; RGD1308555.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 细胞生物 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, Dog, Pig, Horse, Rabbit,
- 产品应用
- WB=1:50-200 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200 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
- 77kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human LCA5
- 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:
Leber congenital amaurosis (LCA) is one of the most common causes of hereditary blindness or severe visual impairment in infants. Mutations in several genes with diverse functions mapping to two loci have been implicated in LCA causation. These proteins are involved in processes such as photoreceptor development and maintenance, phototransduction, vitamin A metabolism and protein trafficking. LCA5, also known as Lebercilin, is a ciliary protein that is widely expressed during development and localizes to the connecting cilia of photoreceptors and to the microtubules, centrioles and primary cilia of cultured mammalian cells. The Leber congenital amaurosis 5-like protein (LCA5L) is a 670 amino acid protein that belongs to the LCA5 family.
Function:
Might be involved in minus end-directed microtubule transport.
Subunit:
Interacts with NINL. Interacts with OFD1.
Subcellular Location:
Cytoplasm, cytoskeleton. Cell projection, cilium axoneme. Cell projection, cilium basal body. Note: In non ciliated cells, localizes to the centrosome and its associated microtubule array.
DISEASE:
Defects in LCA5 are the cause of Leber congenital amaurosis type 5 (LCA5) [MIM:604537]. 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.
Similarity:
Belongs to the LCA5 family.
Database links:Entrez Gene: 167691 Human
Entrez Gene: 75782 Mouse
Entrez Gene: 300866 Rat
Omim: 611408 Human
SwissProt: Q86VQ0 Human
SwissProt: Q80ST9 Mouse
SwissProt: Q5U2Y9 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.