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 PAX-6 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, 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 PAX-6 antibody.

Store the PAX-6 antibody at 2-8oC (with azide) or aliquot and store at -20oC or colder (without azide).