LCA5/Lebercilin antibody

供应商 ：上海康朗生物科技有限公司

库存 ：大量

目录编号 ：kl-9973R

克隆性 ：多克隆

抗原来源 ：Rabbit

保质期 ：12个月

抗体英文名 ：LCA5/Lebercilin antibody

抗体名 ：致盲基因LCA5蛋白抗体

宿主 ：Rabbit

适应物种 ：Human, Mouse, Rat, Dog, Pig, Horse, Rabbit,

免疫原 ：KLH conjugated synthetic peptide derived from human LCA5:121-220/697

亚型 ：IgG

形态 ：冻干粉或液体

应用范围 ：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

浓度 ：1mg/ml

保存条件 ：-20 °C

LCA5/Lebercilin antibody

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/1380元 购买    200ul/2200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.