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.

Suitable for use in FLISA, Western Blot and Immunohistochemistry. Other applications not tested.

Immunohistochemistry (Formalin fixed paraffin embedded): 3ug/ml Optimal dilutions to be determined by the researcher.

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Store product at 4°C in the dark. DO NOT FREEZE! Stable at 4°C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: MaxLight™550 conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Note: Applications are based on unconjugated antibody.

Full-length recombinant corresponding to aa1-385 from AIPL1 (AAH12055) with GST tag. MW of the GST tag alone is 26kD.

Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with MaxLight™550.

Purified by Protein A affinity chromatography.

Recognizes human AIPL1.