This article reviews the emerging evidence linking mitochondrial dysfunction in the pathogenesis and progression of neurodegeneration in select lysosomal storage diseases (LSDs), including GM1-gangliosidosis, mucopolysaccharidosis IIIC, multiple sulfatase deficiency, Krabbe disease, Gaucher disease, Niemann-Pick disease type C and the neuronal ceroid lipofuscinosis. Experimental therapies aimed at restoring mitochondrial function...