Indeed, PD-linked recessive mutations in PINK1 and Parkin are associated with mitochondrial dysfunction and defects in mitophagy, indicating that such mitochondrial pathogenesis can drive the PD phenotype (Matsumine et al., 1997; Hattori et al., 1998; Kitada et al., 1998; Bentivoglio et al., 2001; Valente et al., 2001, 2002). The gene discussed is PRKN; the disease is Parkinson disease.