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Model of CHCHD2/10 heterodimerization in response to loss of Δψ. In well-coupled mitochondria (green) with high Δψ CHCHD2 (C2) is unstable and CHCHD10 (C10) is primarily monomeric. In contrast, in impaired mitochondria with low Δψ, CHCHD2 is stabilized and CHCHD2/10 heterodimerization promoted. CHCHD2 containing the T61I patient mutation (red) incorporates into the heterodimer similarly to WT protein, suggesting a possible mechanism for a dominant effect on CHCHD10 function.
