Proteins in this entry contain WD40 repeats, and include WD repeat-containing proteins 19 (WDR19), from mammals, and dyf-2 from nematodes. Both WDR19 and dyf-2 are components of the IFT complex A (IFT-A), which is required for retrograde ciliary transport [, ]. Mutations in the WDR19 gene are associated with numerous diseases, including cranioectodermal dysplasia 4, short-rib thoracic dysplasia 5 with or without polydactyly, nephronophthisis 13 [], and Senior-Loken syndrome 8 [].
This entry represents the WD-40 repeat found in WDR19 and its homologue, dyf-2. They are part of the IFT complex A (IFT-A), a complex required for retrograde ciliary transport [].WD-40 repeats (also known as WD or beta-transducin repeats) are short ~40 amino acid motifs, often terminating in a Trp-Asp (W-D) dipeptide. WD40 repeats usually assume a 7-8 bladed β-propeller fold, but proteins have been found with 4 to 16 repeated units, which also form a circularised β-propeller structure. WD-repeat proteins are a large family found in all eukaryotes and are implicated in a variety of functions ranging from signal transduction and transcription regulation to cell cycle control and apoptosis. Repeated WD40 motifs act as a site for protein-protein or protein-DNA interaction, and proteins containing WD40 repeats are known to serve as platforms for the assembly of protein complexes or mediators of transient interplay among other proteins []. The specificity of the proteins is determined by the sequences outside the repeats themselves. Examples of such complexes are G proteins (beta subunit is a β-propeller), TAFII transcription factor, and E3 ubiquitin ligase [, ]. In Arabidopsis spp., several WD40-containing proteins act as key regulators of plant-specific developmental events.
