Figure 2

A tRNA mutation disrupts respiratory chain complex I in renal oncocytoma, whereas BHD oncocytomas have intact mitochondria. Immunohistochemistry for complex I (NDUFB8; a and b), complex II (SHDA; c and d), and complex IV (COXIV; e and f) in renal oncocytoma sample with heteroplasmic tRNA mutation compared with BHD oncocytoma shows absence of CI staining in oncocytoma with tRNA mutation (a), but presence of CI staining in BHD oncocytoma (b). CII and CIV staining is present in both tumors (magnification: × 100). (g), Complex I activity assay confirms very low activity in BMF oncocytomas, compared with normal kidney tissue activity, but similar CI activity between BHD tumors and normal kidney tissue.