
Formalin-fixed, paraffin-embedded human tonsil stained with PAX3 Mouse Monoclonal Antibody (PAX3/8424). Inset: PBS instead of primary antibody; secondary only negative control.
Pax genes contain paired domains that share strong homology to genes in Drosophila which are involved in programming early development. The product of the PAX3 gene is a DNA-binding protein expressed during early neurogenesis. Pax-3 is a protein containing both a paired domain and a paired-type homeodomain. During early neurogenesis, Pax-3 expression is limited to mitotic cells in the ventricular zone of the developing spinal cord and to distinct regions in the hindbrain, midbrain and diencephalon. In 10-12 day embryos, expression of Pax-3 is also seen in neural crest cells of the developing spinal ganglia, the craniofacial mesectoderm and in limb mesenchyme. Mutations in the MITF and Pax-3 genes, encoding transcription factors, are responsible for Waardenburg syndrome II (WSII) and WSI/WSIII, respectively.
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