Figure 1. Identification of the D122FS mutation in an ARS patient.
A. Pedigree of the affected proband showing the D122FS mutation. B. Sequence of the PITX2 fragment from genomic DNA of unaffected and affected members of the family. Deletion of a single base (C) results in a codon frameshift that predicts a truncated protein. C. Schematic representation of the human PITX2a gene showing the relative positions of the homeodomain (HD), the 2 C-terminal mutations, and the 14 amino acid Otp and aristaless homologous region (OAR) (Cox et al., 2002).