Figure 1.

Mutations of the respiratory chain components in NMDs. The five complexes of the respiratory chain are schematized, and the various mutated subunits found in NMDs are reported. mtDNA-encoded subunits are highlighted in blue, while nDNA-encoded subunits are in red. Assembly factors that are not part of the mature complex are indicated in green. The structures of Complexes I–V were obtained from the Protein Data Bank (PDB) (http://www.rcsb.org/, accessed on 13 September 2021) with the accession codes PBD ID: 5LNK (Complex I), 1NEN (Complex II), 6FO0 (Complex III), 5Z62 (Complex IV), and 2XND (Complex V). For details see the text.