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. 2013 Jul 9;8(7):e69003. doi: 10.1371/journal.pone.0069003

Figure 2. A new structural map of human mitochondrial disease mutation sites in Complexes III and IV.

Figure 2

Mitochondrial diseases result in diverse pathology, and can be multi-systemic or tissue-specific. Here we show 93 individual mutation sites mapped onto their corresponding 3D crystal structures. Each mutation site is shown as a sphere colored according to the primary pathology or tissue (see legend) found to be affected in either single or groups of patients. The complete dimeric Complex IV is depicted as a ribbon model (A) with the mitochondrial-encoded subunits, MT-CO1 (B), MT-CO2 (C) and MT-CO3 (D), colored orange, yellow and green, respectively. The three COX monomers are shown separately for clarity. The dimeric Complex III is shown in the same format (E) with the mitochondrial-encoded MT-CYB (F) subunits colored as blue ribbons.