In accord with our observations in MEFs lacking neurofibromin, NDI1 expression increases OCR (Fig. 5A, B) and reduces tumorigenicity (Fig. 5C) in MPNST cells too, and a similar antineoplastic effect is obtained by SIRT3 overexpression (Fig. 5D, E). The gene discussed is AVPR2; the disease is malignant peripheral nerve sheath tumor.