Notably, CNGA3 and CNGB3 function together by forming a heterotetramer cyclic nucleotide-gated (CNG) channel resulting in up to 95% of achromatopsia patients carrying pathogenic defects in the CNGA3/CNGB3 channel complex (cone CNG channel) [6, 7]. Here, CNGA3 is linked to achromatopsia.