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. 2022 Mar 30;42(13):2631–2646. doi: 10.1523/JNEUROSCI.1459-21.2022

Figure 6.

Figure 6.

Accumulation of saccharopine in Aass (G489E) mice do not affect mitochondrial function. A, TEM images of mitochondria in the brains of WT, Aass (G489E), and Aass (R65Q) mice. Scale bars: 1 μm. B, C, The average sizes of mitochondria in brains of WT, Aass (G489E) (B, Student's t test, p = 0.5065) and Aass (R65Q) mice (C, Student's t test, p = 0.2993). WT, 517 mitochondria, n = 4 brains; Aass (G489E), 404 mitochondria, n = 3 brains; Aass (R65Q), 181 mitochondria, n = 3 brains. D–G, Representative profile of mitochondria stress test showing the OCR of the cultured neurons isolated from WT, Aass (G489E) (D), or Aass (R65Q) (F) cortex. Graphs showing basal respiration, protein leak, ATP production, nonmitochondrial oxygen, max respiration and spare respiration of cortex neurons of Aass (G489E) (E) or Aass (R65Q) (G) mice compared with cortex neurons of WT littermates. WT, n = 3 brains; Aass (G489E), n = 3 brains; Aass (R65Q), n = 3 brains. Data are presented as mean ± SEM, n.s., no significance, p > 0.05.