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| Reactivity: | Human, Mouse, Rat |
| Applications: | WB, IF/IC, ELISA |
| Host Species: | Rabbit |
| Clonality: | Polyclonal antibody |
| Gene Name: | AIP like 1 HSP90 co-chaperone |
| Gene Symbol: | AIPL1 |
| Synonyms: | LCA4; AIPL2; AIPL1 |
| Gene ID: | 23746 |
| UniProt ID: | Q9NZN9 |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-384 of human AIPL1 (NP_055151.3). |
| Dilution: | WB 1:500-1:2000; IF/IC 1:50-1:200 |
| Purification Method: | Affinity purification |
| Concentration: | 1.00 mg/ml |
| Buffer: | PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Storage: | Store at -20°C. Avoid freeze/thaw cycles. |
| Documents: | Manual-AIPL1 antibody |
Background
Leber congenital amaurosis (LCA) is the most severe inherited retinopathy with the earliest age of onset and accounts for at least 5% of all inherited retinal diseases. Affected individuals are diagnosed at birth or in the first few months of life with nystagmus, severely impaired vision or blindness and an abnormal or flat electroretinogram. The photoreceptor/pineal-expressed gene, AIPL1, encoding aryl-hydrocarbon interacting protein-like 1, is located within the LCA4 candidate region. The encoded protein contains three tetratricopeptide motifs, consistent with chaperone or nuclear transport activity. Mutations in this gene may cause approximately 20% of recessive LCA. Alternative splicing results in multiple transcript variants.
Images
 | Western blot analysis of various lysates using AIPL1 Rabbit pAb (A14113) at 1:1000 dilution. Secondary antibody: HRP-conjugated Goat anti-Rabbit IgG (H+L) (AS014) at 1:10000 dilution. Lysates/proteins: 25μg per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Enhanced Kit (RM00021). Exposure time: 90s. |
 | Immunofluorescence analysis of MCF7 cells using AIPL1 Rabbit pAb (A14113). Secondary antibody: Cy3-conjugated Goat anti-Rabbit IgG (H+L) (AS007) at 1:500 dilution. Blue: DAPI for nuclear staining. |
 | Immunofluorescence analysis of Y79 cells using AIPL1 Rabbit pAb (A14113) at dilution of 1:100. Blue: DAPI for nuclear staining. |
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