Figure 7.

A HBSO model of CCHS

(A) Schematic showing the establishment of hPSC-based model of CCHS.

(B) Electrographs of Sanger sequencing of PHOX2B exon 3 showing the “correction” of +7Ala-PARM in PHOX2B locus.

(C) Immunostaining of PHOX2B and ISL1 in the day-60 15C11 and 15C11-C HBSOs. Arrowheads mark the PHOX2B⁺ISL1⁺ neurons.

(D) Immunostaining of PHOX2B, TH, and VGLUT2 in the day-60 15C11 and 15C11-C HBSOs. The putative RTN (PHOX2B⁺VGLUT2⁺TH⁻) and PHOX2B⁺ TH⁺ neurons are marked by filled and open arrowheads, respectively.

(E–G) Bar charts showing the percentages of (E) PHOX2B⁺, (F) PHOX2B⁺VGLUT2⁺, and (G) PHOX2B⁺TH⁺ cells in the day-60 15C11 and 15C11-C HBSOs. Data are represented as mean ± SEM. n ≥ 6 per group from three independent experiments. Unpaired t test, two-sided. See also Figure S7.