α-ketoglutarate dehydrogenase complex (KGDHC) activity is reduced as a consequence of fumarate derived protein succination.A-B, Quantification of fumarate (A, n = 4–5) and lactate (B, n = 5–6) content in the olfactory bulb of Ndufs4 KO and WT mice. C-D, KGDHC activity in the olfactory bulb (C, n = 4) and brainstem (D, (n = 3–4) of Ndufs4 KO and WT mice. E-F, Quantification of L-2-hydroxyglutarate (E, n = 5–6) and α-ketoglutarate (F, n = 4–5) content in the olfactory bulb of Ndufs4 KO and WT mice. G-H, Mitochondrial total ATP synthesis (no oligomycin) and substrate level phosphorylation (SLP, +8 μM oligomycin = O) in the olfactory bulb (G, n = 5–6) and the brainstem (H, n = 4) in the presence of 5 mM α-ketoglutarate (αKG) as respiratory substrate. I-J, Same as in G-H but using 5 mM succinate (SUC) as respiratory substrate (n = 7–12).In panels A-J, results expressed as mean ± SEM, and comparisons performed by unpaired t-test (*p < 0.05, **p < 0.01 and ***p < 0.001 vs. WT).