CEP290 and ciliopathy: Additional loss of Bbs4 alleles in Cep290rd16-mutant (a hypomorphic allele that contains an internal in-frame deletion of 1599–1897 amino acids from the CEP290 protein) mice increases their body weight and accelerates photoreceptor degeneration compared with Cep290rd16 single homozygous mutant mice, suggesting a modifying role for the BBSome in CEP290 ciliopathy phenotypes (Zhang et al., 2014).