Fig. 2.

Structural modelling of reported SDHA missense variants onto the reported mammalian crystal structure of complex II (PDB: 1ZOY). The pathogenic missense variants, p.(Thr508Ile), p.(Ser509Leu), p.(Arg554Trp) and p.(Gly555Glu) variants are situated at the interface between the SDHA and SDHB subunits. Recessive SDHA variants are shows in black text, whilst the dominant p.(Arg451Cys) SDHA variant is shown in blue. The SDHA protein is shown in grey, whilst the SDHB protein is shown in green; the three iron‑sulphur clusters within SDHB are shown as orange clusters. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)