Unlike many recessive loss-of-function mutations identified in classical cilia-related genes, e.g., DNAH and NKE family members, most pathogenic NUP mutations (excluding NUP188) in patients with SRNS or CHD are missense mutations (Miyake et al., 2015; Braun et al., 2016; Braun et al., 2018; Chen et al., 2019; Muir et al., 2020; Chen et al., 2022b). Here, NUP188 is linked to coronary artery disorder.