Anti-LCA5/Lebercilin抗体-抗体-抗体-生物在线
Anti-LCA5/Lebercilin抗体

Anti-LCA5/Lebercilin抗体

商家询价

产品名称: Anti-LCA5/Lebercilin抗体

英文名称: LCA5/Lebercilin

产品编号: YB--9973R

产品价格: null

产品产地: 中国/美国

品牌商标: Ybscience

更新时间: 2023-08-17T10:29:50

使用范围: 科研使用

上海钰博生物科技有限公司
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 Anti-LCA5/Lebercilin抗体

产品编号 YB-9973R
英文名称 LCA5/Lebercilin
中文名称 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/1580元 购买    200ul/2480元 购买    大包装/询价
说 明 书 100ul  200ul
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=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 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 77kDa
细胞定位 细胞浆 
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human LCA5:121-220/697 
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.
PubMed PubMed
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.

SWISS:
Q86VQ0 

Gene ID:
167691

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