Figure 2.

Maturation of human WT cones following transplantation as a purified cell suspension into the rd1/FoxN1^nu model of advanced degeneration

(A) Quantification of percentage of cells in SRS that were MOPSIN⁺ and formed segment-like structures as a proportion of HNA⁺ nuclei found within the cell mass (81% ± 6% MOPSIN⁺ cells versus 82% ± 2%, WT versus CNGB3, no significant [N.S.] differences between WT versus CNGB3, Mann-Whitney test; mean ± SEM). A proportion of these cells displayed MOPSIN localized to nascent segment-like structures (17% ± 3% versus 13% ± 1%, WT versus CNGB3; N.S., Mann-Whitney test).

(B) GFP⁺ cells (green) expressing hCARR (white) and MOPSIN (red), which localized to nascent segment-like structures in some cells (ROI1, arrows). Rare host M-cones were identified, but no segment-like structures were associated with these cells (ROI2, arrowhead).

(C) Numerous PRPH2⁺ (red) segment-like structures (arrows) can be seen within the cell mass. ROI1–3, single confocal sections through some segment-like structures.

(D) Human (h) mitochondria-rich (white) structures were in close proximity to PRPH⁺ structures (ROI and dual channel).

(E) Quantification of number of PRPH2⁺ segment-like structures as proportion of nuclei found within the cell mass (25% ± 3% versus 23% ± 4%, WT versus CNGB3; N.S., Mann-Whitney test; mean ± SEM, n > 5 images, n > 5 retinas per group).

(F and G) Representative TEM images of structures consistent with mitochondria-rich ISs and stacked disks of OSs (ROI).

Scale bars, 25 μm (A–D); 12.5 μm (ROIs in B and D); 0.5 μm (ROI in F and G). IS, inner segment; OS, outer segment.