Schematic representation of Phox2bwt and mutant expression constructs (A) and cellular localization (B) of Phox2wt, Phox2bR100L, Phox2bR141G, Phox2bK155X, Phox2b676delG and Phox2HDen. A, Scheme illustrating the Phox2b structure, the location of the homeodomain and polyalanine repeats (plain boxes) and of Phox2b mutations (red). The fusion construct between the homeodomain and the engrailed repressor (Phox2HDen) is also shown schematically. B, Phox2b expression plasmids were transfected in DF1-cells and the expression of Phox2bwt and Phox2b variants was analyzed by RT-PCR and immunostaining, using an antibody directed against the Phox2 HD. All Phox2b variants were detected at RNA and protein level. Scale bar, 10 μm.