Based on the information provided in the URL, the AIPL1 Polyclonal Antibody (CAB6458) is designed for research involving AIPL1, a protein associated with Leber congenital amaurosis (LCA), a genetic eye disorder that causes severe vision loss. This antibody, produced in rabbits, is highly specific for human samples and is validated for Western blot applications.AIPL1 is a key protein involved in the maintenance and function of photoreceptor cells in the retina, making it essential for normal vision. Mutations in the AIPL1 gene are known to cause LCA, highlighting the importance of studying this protein for understanding the mechanisms underlying this vision impairment disorder.
By targeting AIPL1 with this polyclonal antibody, researchers can detect and analyze the levels and localization of AIPL1 in various cells and tissues, providing valuable insights into its role in vision and potential therapeutic strategies for treating LCA. Overall, the AIPL1 Polyclonal Antibody (CAB6458) offers a valuable tool for advancing research in the field of ophthalmology and genetic eye disorders.
Product Name:
AIPL1 Rabbit Polyclonal Antibody
SKU:
CAB6458
Size:
20uL, 100uL
Isotype:
IgG
Host Species:
Rabbit
Reactivity:
Human,Mouse
Immunogen:
Recombinant fusion protein containing a sequence corresponding to amino acids 1-384 of human AIPL1 (NP_055151.3).
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.
Purification Method:
Affinity purification
Gene ID:
23746
Storage Buffer:
Store at -20℃. Avoid freeze / thaw cycles.Buffer: PBS with 0.02% sodium azide,50% glycerol,pH7.3.
Western blot analysis of extracts of various cell lines, using AIPL1 antibody (CAB6458) at 1:1000 dilution.Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution.Lysates/proteins: 25μg per lane.Blocking buffer: 3% nonfat dry milk in TBST.Detection: ECL Basic Kit (AbGn00020).Exposure time: 90s.
