Human Aryl Hydrocarbon Interacting Protein Like 1 (AIPL1) ELISA Kit
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Description
Human Aryl Hydrocarbon Interacting Protein Like 1 (AIPL1) ELISA Kit is an ELISA Kit for the in vitro quantitative measurement of Human Aryl Hydrocarbon Receptor Interacting Protein Like Protein 1 (AIPL1) concentrations in tissue homogenates, cell lysates and other biological fluids.
Documents del producto
Product specifications
| Category | ELISA Kits |
| Immunogen Target | Aryl Hydrocarbon Interacting Protein Like 1 (AIPL1) |
| Reactivity | Human |
| Detection Method | Colorimetric |
| Assay Data | Quantitative |
| Assay Type | Sandwich |
| Test Range | 0.313 ng/ml - 20 ng/ml |
| Sensitivity | < 0.19 ng/ml |
| Recommended Dilution | Optimal dilutions/concentrations should be determined by the end user. |
| Size 1 | 96 tests |
| Size 2 | 5 × 96 tests |
| Size 3 | 10 × 96 tests |
| Form | Lyophilized |
| Tested Applications | ELISA |
| Sample Type | Tissue homogenates, cell lysates and other biological fluids. |
| Availability | Shipped within 5-12 working days. The validity for this kit is at least 6 months. Up to 12 months validity can be provided on request. |
| Storage | Shipped at 4 °C. Upon receipt, store the kit according to the storage instruction in the kit's manual. |
| Dry Ice | No |
| UniProt ID | Q9NZN9 |
| Gene ID | 23746 |
| OMIM | 604392 |
| Alias | AIPL2, LCA4 |
| Background | Elisa kits for AIPL1 |
| Status | RUO |
| Note | Validity: The validity for this kit is at least 6 months. Up to 12 months validity can be provided on request. This product is for research use only. The range and sensitivity is subject to change. Please contact us for the latest product information. For accurate results, sample concentrations must be diluted to mid-range of the kit. If you require a specific range, please contact us in advance or write your request in your order comments. Please note that our ELISA and CLIA kits are optimised for detection of native samples, rather than recombinant proteins. We are unable to guarantee detection of recombinant proteins, as they may have different sequences or tertiary structures to the native protein. |
Descripción
AIPL1 is a specialized molecular chaperone predominantly expressed in retinal photoreceptor cells, where it plays a crucial role in the folding, stability, and assembly of proteins essential for phototransduction. Structurally, AIPL1 contains tetratricopeptide repeat (TPR) domains, which mediate protein-protein interactions, and a FKBP-like domain, enabling its chaperone activity. AIPL1 is critical for the proper function of phosphodiesterase 6 (PDE6), a key enzyme in the visual cycle. It helps stabilize PDE6 subunits during their assembly, ensuring efficient phototransduction signaling. Mutations in the AIPL1 gene are associated with severe retinal degenerative diseases, including Leber congenital amaurosis type 4 (LCA4) and retinitis pigmentosa (RP). These conditions result from impaired PDE6 function, leading to photoreceptor cell death and progressive vision loss. Due to its specific expression in photoreceptors and essential role in their survival, AIPL1 is a target of interest for gene therapy and retinal disease research.
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AIPL1 Antibody is a Rabbit Polyclonal antibody against AIPL1. 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.
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