Pax genes contain paired domains with strong homology to genes in Drosophila, which are involved in programming early development. Lesions in the Pax-6 gene account for most cases of aniridia, a congenital malformation of the eye, chiefly characterized by iris hypoplasia, which can cause blindness. Pax-6 is involved in other anterior segment malformations besides aniridia, such as Peters anomaly, a major error in the embryonic development of the eye with corneal clouding with variable iridolenticulocorneal adhesions. The Pax-6 gene encodes a transcriptional regulator that recognizes target genes through its paired-type DNA-binding domain. The paired domain is composed of two distinct DNA-binding subdomains, the amino-terminal subdomain and the carboxy-terminal subdomain, which bind respective consensus DNA sequences. The human Pax-6 gene produces two alternatively spliced isoforms that have the distinct structure of the paired domain.

Optimal dilution of the PAX6 antibody should be determined by the researcher.

1. Staining of formalin-fixed tissues requires boiling tissue sections in 10mM Tris buffer with 1mM EDTA, pH 9.0, for 10-20 min followed by cooling at RT for 20 min2. The prediluted format is supplied in a dropper bottle and is optimized for use in IHC. After epitope retrieval step (if required), drip mAb solution onto the tissue section and incubate at RT for 30 min.

A recombinant fragment (N-terminus; aa 1-300) was used as the immunogen for the PAX6 antibody.

Store the PAX6 antibody at 2-8oC (with azide) or aliquot and store at -20oC or colder (without azide).