Mitochondrial Dysfunction Models

1 Reactive Oxygen Species Production and Damage

Reactive oxygen species (ROS) are an unavoidable byproduct of electron transport. Superoxide (\(\text{O}_2^{\bullet-}\)) is generated primarily at Complexes I and III when electrons “leak” to molecular oxygen instead of following the normal transfer chain. The ROS production rate increases when electron flow is impeded:

\[ J_{\text{ROS}} = k_{\text{ROS}} \cdot [\text{NADH}] \cdot (1 - \frac{J_{\text{CI}}}{J_{\text{CI,max}}}) + k_{\text{ROS,CIII}} \cdot [\text{UQH}_2] \cdot (1 - \frac{J_{\text{CIII}}}{J_{\text{CIII,max}}}) \tag{1}\]

where the terms \((1 - J / J_{max})\) capture the principle that reduced electron flow (due to impaired complexes) leads to increased electron residence time on carriers, increasing the probability of electron leak to oxygen. This creates a positive feedback loop: ETC damage \(->\) reduced flux \(->\) increased ROS \(->\) further ETC damage. The ROS balance includes antioxidant scavenging:

\[ \frac{d[\text{ROS}]}{d t} = J_{\text{ROS}} - k_{\text{SOD}} \cdot [\text{SOD}] \cdot [\text{ROS}] - k_{\text{GPx}} \cdot [\text{GPx}] \cdot [\text{ROS}] \tag{2}\]

where SOD (superoxide dismutase) and GPx (glutathione peroxidase) are the primary antioxidant enzymes. Oxidative damage accumulates as a function of cumulative ROS exposure, reducing enzyme activities over time and establishing the vicious cycle described qualitatively in Energy Metabolism and Mitochondrial Function.

2 Impaired Energy Production in ME/CFS

The ME/CFS disease state is modeled by modifying healthy-state parameters at specific points supported by experimental evidence:

  • Reduced Complex I activity: \(V_{max, \text{CI}}^{\text{ME/CFS}} = \alpha_{\text{CI}} \cdot V_{max, \text{CI}}^{\text{healthy}}\) with \(\alpha_{\text{CI}} \in [0.5, 0.8]\), consistent with findings of impaired Complex I function in ME/CFS lymphocytes (Tomas et al. 2017)
  • Increased proton leak: \(J_{\text{leak}}^{\text{ME/CFS}} = \beta \cdot J_{\text{leak}}^{\text{healthy}}\) with \(\beta \in [1.5, 3.0]\), reducing the fraction of proton-motive force available for ATP synthesis
  • Reduced \(\text{NAD}^\text{+}\) pool: \([\text{NAD}^+]_{\text{total}}^{\text{ME/CFS}} = \gamma \cdot [\text{NAD}^+]_{\text{total}}^{\text{healthy}}\) with \(\gamma \in [0.6, 0.9]\), consistent with abnormal NAD+ metabolism documented in ME/CFS white blood cells (Heng et al. 2025) (note: Heng 2025 found NAD+ elevated in PBMCs, suggesting substrate backup; the \(\gamma < 1\) assumption applies to energy-intensive tissues where depletion is predicted but not yet measured)
  • Elevated baseline ROS: \([\text{ROS}]_0^{\text{ME/CFS}} > [\text{ROS}]_0^{\text{healthy}}\), consistent with oxidative stress biomarkers

The model predicts that these parameter changes produce a new steady state with reduced ATP production, elevated lactate, and increased sensitivity to metabolic perturbation—qualitatively matching the ME/CFS metabolic phenotype described by Naviaux (Naviaux et al. 2016) and Germain et al. (Germain et al. 2020).

WarningLimitation: Parameter Uncertainty in Energy Models

The impairment factors (\(\alpha_{\text{CI}}\), \(\beta\), \(\gamma\)) are constrained by in vitro studies on isolated cells, primarily lymphocytes and muscle biopsy samples. Whether these values accurately represent in vivo mitochondrial function across tissues remains uncertain. Patient-level variation in these parameters likely contributes to the clinical heterogeneity of ME/CFS.

References

Germain, Arnaud, Dinesh K Barupal, Susan M Levine, and Maureen R Hanson. 2020. “Comprehensive Circulatory Metabolomics in ME/CFS Reveals Disrupted Metabolism of Acyl Carnitines and Fatty Acids.” Metabolites 10 (1): 34. https://doi.org/10.3390/metabo10010034.
Heng, Ruiwen Benjamin, Bavani Gunasegaran, Shivani Krishnamurthy, Sonia Bustamante, Ananda Staats, Sharron Chow, Seong Beom Ahn, et al. 2025. “Mapping the Complexity of ME/CFS: Evidence for Abnormal Energy Metabolism, Altered Immune Profile, and Vascular Dysfunction.” Cell Reports Medicine 6 (12): 102514. https://doi.org/10.1016/j.xcrm.2025.102514.
Naviaux, Robert K., Jane C. Naviaux, Kefeng Li, A. Taylor Bright, William A. Alaynick, Lin Wang, Asha Baxter, Neil Nathan, Wayne Anderson, and Eric Gordon. 2016. “Metabolic Features of Chronic Fatigue Syndrome.” Proceedings of the National Academy of Sciences 113 (37): E5472–80. https://doi.org/10.1073/pnas.1607571113.
Tomas, Cara, Andreas Finkelmeyer, Tim Hodgson, Laura MacLachlan, Guy A. MacGowan, Andrew M. Blamire, and Julia L. Newton. 2017. “Elevated Brain Natriuretic Peptide Levels in Chronic Fatigue Syndrome Associate with Cardiac Dysfunction: A Case Control Study.” Open Heart 4 (2): e000697. https://doi.org/10.1136/openhrt-2017-000697.