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. 2016 May 16;6:26013. doi: 10.1038/srep26013

Figure 4. Assessment of mitochondrial respiratory chain deficiency in Purkinje cell neurons in human cerebellum.

Figure 4

250 μm-thick cerebellum section were passively cleared and immunofluorescently labelled to identify mitochondrial mass (porin and SDHA, 647 nm) and complex I subunits within the mitochondrial respiratory chain (NDUFB8 and NDUFA13; 546 nm) in conjunction with a neuronal marker (NF-H; 488 nm) in control 1 (a). To interrogate respiratory chain deficiency (reduced abundance of a complex I subunit relative to mitochondrial mass) in Purkinje cells and their projections in patients with mitochondrial disease neurons (NF-H; 488 nm), mitochondria (porin; 647 nm) and complex I subunit (NDUFB8; 546 nm) were immunofluorescently labelled. Control Purkinje cells reveal matched abundance of NDUFB8 protein relative to porin protein (control 1 and 2), while patient Purkinje cells and surrounding neurons showed an absence of NDUFB8 protein relative to porin protein level confirming the presence of complex I deficient mitochondria in these cells (patient 2 and 4; (b)). Scale: 100 μm.