BCS1L and Aminoaciduria: For example, point mutations of the Complex III chaperone ubiquinol-cytochrome c reductase complex chaperone (BCS1L) can cause the rare hereditary disease GRACILE (Growth Retardation, Aminoaciduria, Cholestasis, Iron overload, Lactic acidosis, and Early death) syndrome (Visapää et al., 2002; Fellman et al., 1998; Fellman, 2002; Fellman et al., 2008; Levéen et al., 2011).