RPE65 Antibody Blocking Peptide (合成多肽) | Bioss

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货号：bs-9575P
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概述

产品编号
bs-9575P
英文名称
RPE65 Antibody Blocking Peptide
中文名称
视网膜色素上皮细胞特异性蛋白65封闭多肽
英文别名
All-trans-retinyl-palmitate hydrolase; LCA 2; LCA2; Leber congenital amaurosis; mRPE 65; mRPE65; p63; rd 12; rd12; Retinal pigment epithelium specific 61 kDa protein; Retinal pigment epithelium specific 65 kDa protein; Retinal pigment epithelium specific protein; Retinal pigment epithelium specific protein 65kDa; Retinal pigment epithelium-specific 65 kDa protein; Retinitis pigmentosa 20; Retinoid isomerohydrolase; RP 20; RP20; RPE 65; RPE65; RPE65_HUMAN; sRPE 65; sRPE65.
纯化方法
HPLC
研究领域

Metabolism > Pathways and Processes > Cofactors, Vitamins / minerals > Vitamins / minerals

Neuroscience > Sensory System > Visual system

Signal Transduction > Metabolism > Vitamins / Minerals

Tags & Cell Markers > Cell Type Markers > Neuroscience Markers > Sensory Neurons

活性
Not tested
保存条件
Shipped at 4℃. Stored at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
数据库链接

Entrez Gene : 6121 Human

Entrez Gene : 19892 Mouse

Entrez Gene : 89826 Rat

Omim : 180069 Human

SwissProt : Q9YGX2 Chicken

SwissProt : Q28175 Cow

SwissProt : Q16518 Human

SwissProt : Q91ZQ5 Mouse

SwissProt : O70276 Rat

Unigene : 2133 Human

Unigene : 131708 Mouse

Unigene : 76724 Rat

背景资料
The retinal pigment epithelium (RPE) is a monolayer simple epithelium in proximity to the outer surface of the retinal photoreceptor cells. Retinal pigment epithelium-specific protein (RPE65) is a 65 kDa protein belonging to the β-carotene dioxygenase family. This protein is important in 11-cis retinal production as well as in visual pigment regeneration. RPE65 is attached to the membrane by a lipid anchor when palmitoylated (membrane form) and soluble when unpalmitoylated. The soluble form of the protein binds vitamin A. Defects in RPE65 causes autosomal dominant retinitis pigmentosa and/or Leber congenital amaurosis type 2.

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靶标

基因名
RPE65
蛋白名
Retinoid isomerohydrolase
亚基
Interacts with MYO7A; this mediates light-dependent intracellular transport of RPE65.
亚细胞定位
Cytoplasm. Cell membrane. Attached to the membrane by a lipid anchor when palmitoylated (membrane form), soluble when unpalmitoylated.
组织特异性
Retinal pigment epithelium specific.
翻译后修饰
Palmitoylation by LRAT regulates ligand binding specificity; the palmitoylated form (membrane form) specifically binds all-trans-retinyl-palmitate, while the soluble unpalmitoylated form binds all-trans-retinol (vitamin A).
疾病
Defects in RPE65 are the cause of Leber congenital amaurosis type 2 (LCA2) [MIM:204100]. 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.
相似性
Belongs to the carotenoid oxygenase family.
功能
Plays important roles in the production of 11-cis retinal and in visual pigment regeneration. The soluble form binds vitamin A (all-trans-retinol), making it available for LRAT processing to all-trans-retinyl ester. The membrane form, palmitoylated by LRAT, binds all-trans-retinyl esters, making them available for IMH (isomerohydrolase) processing to all-cis-retinol. The soluble form is regenerated by transferring its palmitoyl groups onto 11-cis-retinol, a reaction catalyzed by LRAT. The enzymatic activity is linearly dependent of the expression levels and membrane association.

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