Loss of AMP-Activated Protein Kinase Induces Mitochondrial Dysfunction and Proinflammatory Response in Unstimulated Abcd1-Knockout Mice Mixed Glial Cells

Figure 2

Mitochondrial complex subunit expression and levels in healthy control, adrenomyeloneuropathy (AMN), and adrenoleukodystrophy (ALD) patient-derived fibroblasts. Primary patient-derived skin fibroblasts from healthy control (CTL), AMN, and ALD were cultured as described in Section 2. mRNA (a) and protein (b) levels of mitochondrial complex subunits were significantly reduced in ALD patient-derived fibroblasts. (c) Densitometric ratio of mitochondrial subunit levels versus actin in western blots. Results represent the mean ± SD of triplicates from two different experiments. , , and ALD compared with AMN. AMN compared with CTL. COM: complex, NS: nonsignificant.

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