Aryl Hydrocarbon Interacting Protein Like 1 (AIPL1) Antibody
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Description
AIPL1 Antibody is a Rabbit Polyclonal against AIPL1.
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Product specifications
| Category | Primary Antibodies |
| Immunogen Target | Aryl Hydrocarbon Interacting Protein Like 1 (AIPL1) |
| Host | Rabbit |
| Reactivity | Human, Mouse, Rat |
| Recommended Dilution | WB: 1/200 - 1/2000, IHC: 1/20 - 1/200. Optimal dilutions/concentrations should be determined by the end user. |
| Clonality | Polyclonal |
| Conjugation | Unconjugated |
| Isotype | IgG |
| Purity | ≥ 95% (SDS-PAGE) |
| Purification | Purified by immunogen affinity chromatography. |
| Size 1 | 100 µg |
| Form | Liquid |
| Tested Applications | ELISA, WB, IHC |
| Buffer | PBS, pH 7.3, with 0.02% sodium azide and 50% glycerol. |
| Availability | Shipped within 5-12 working days. |
| Storage | Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles. |
| Dry Ice | No |
| UniProt ID | Q9NZN9 |
| Gene ID | 23746 |
| OMIM | 604392 |
| Alias | AIPL2, LCA4 |
| Background | Antibody anti-AIPL1 |
| Status | RUO |
| Note | Concentration: 2 mg/ml - Validity: 12 months. |
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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