Fig. 1. Mutations in sdhA block SDH complex activity.

(A). A schematic illustration of the electron transport chain, complexes I-V. IMS denotes the intermembrane space, IM: inner membrane, M:matrix, Ub: ubiquinone, CytC: cytochrome C. (B) A schematic of the SdhA protein structure and the sequences of identified mutations. (C-E) Eye-specific mosaic 3rd instar larval eye discs visualizing the enzymatic activity of the SDH complex. (C) Wild Type. Chevrons demark the morphogenetic furrow. (D) sdhA mutant. (E) Wild-type disc, stained in the presence of the inhibitor malonate.