Mice carrying an insertion in the mpv17 gene abolishing protein function develop mitochondrial DNA depletion, most prominently in the liver, and late onset glomerulosclerosis, hair graying and neurodegeneration of the peripheral nervous system (Meyer zum Gottesberge et al., 2012; Müller et al., 1997; Viscomi et al., 2009; Weiher et al., 1990). The gene discussed is MPV17; the disease is glomerulosclerosis.