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. Author manuscript; available in PMC: 2012 Aug 1.
Published in final edited form as: J Neuropathol Exp Neurol. 2012 Feb;71(2):148–161. doi: 10.1097/NEN.0b013e318244477d

Figure 4.

Figure 4

High levels of complex I-deficient neurons can be detected in remaining neuronal populations. The inferior olivary nucleus shows an unusual distribution of mitochondria (m.3243A>G, first column) (porin), whereas mitochondria appear abundant throughout the remainder of the cerebellum in all cases as can be seen in the patient with the m.13094T>C mutation (middle column) and a patient with recessive POLG mutation (third column). High levels of complex I-deficient neurons are observed throughout the olivo-cerebellum, nearing 100% of inferior olivary nucleus in m.3243A>G patient but there is marked deficiency throughout the remainder of the cerebellum. Complex IV-deficiency was rarely detected and low in many cases; cytochrome c oxidase/succinic acid dehydrogenase (COX/SDH) histochemistry often revealed intact neuronal COX activity. Scale bar: 100 μm.