遺傳性失明相關蛋白AIPL1抗體（）

英文名稱：      Anti-AIPL1                               濃度： 1mg/1ml 

抗體來源： Rabbit

克隆類型：   polyclonal          

 交叉反應：  Human, Mouse, Rat, Dog, Pig, Cow, Rabbit, Sheep, 

產品類型: 一抗  

性    狀:   Lyophilized or Liquid 

保存條件： 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. 

產品介紹 ：The inherited blindness associated protein, aryl hydrocarbon receptor interacting protein-like 1 （AIPL1）, interacts with the cell cycle regulator protein NUB1. AIPL1 is crucial for protein folding and stabilization, as well as for protein trafficking. It localizes to the nucleus or cytoplasm and is highly expressed in the pineal gland and the retina. In the retina, AIPL1 is expressed in both developing cone and rod photoreceptors, but it is restricted to rod photoreceptors in the adult human retina. Defects in the gene encoding for AIPL1 can cause Leber congenital amaurosis type IV, an early-onset, inherited autosomal recessive disorder that results in childhood blindness.

Function : May be important in protein trafficking and/or protein folding and stabilization.
Subunit : Interacts with NUB1. 
Subcellular Location : Cytoplasm. Nucleus.
Tissue Specificity : Highly expressed in retina. Specifically localized to the developing photoreceptor layer and within the photoreceptors of the adult retina.
DISEASE : Defects in AIPL1 are the cause of Leber congenital amaurosis type 4 （LCA4） [MIM:604393]. 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 : Contains 1 PPIase FKBP-type domain.
Contains 3 TPR repeats.
Database links : UniProtKB/Swiss-Prot: Q9NZN9.2

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