analysis of the protein structure of LRS1. (A) Position of the annotated Pfam domains with the C3HC4 type zinc finger (RING) domain (Pfam:00097) at the N-terminus (blue), two repeats of a WD40 domain (Pfam:00400) at the C-terminal end (orange), the position of the two ß-propeller domains predicted by the model as shown in B (purple and green) and the position of the mutation in hit1 (red) and hit2 (blue). (B)
Ab initio model based on the amino acid sequence of the protein generated with I-TASSER. The RING domain is colored in blue, the seven-fold propeller in purple and the eight-fold propeller harboring the mutations in green, using the DeepView-Swiss-PdbViewer. (C) Top view of the eight-fold propeller structure with the localization of the annotated WD40 repeats (orange) and the two mutations identified in hit1 (red arrow) and hit2 (blue arrow).