Exploring mitochondrial dysfunction linked to Leigh Syndrome by proteome-wide interactome analysis using cross-linking mass spectrometry

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Leigh syndrome (LS) is a rare neurodegenerative disorder with onset in infancy, characterized by delayed psychomotricity and various neurological symptoms. It is associated with a dysfunctional oxidative phosphorylation system, being defects in complex IV (cytochrome-c oxidase, COX) the most common cause. A rarer form of COX-deficient LS, the French Canadian type of LS, is caused by mutations in the LRPPRC gene. The physiological impact of this mutation is not fully understood.
To explore the molecular mechanisms underlying this LS type, we used cross-linking mass spectrometry (XL-MS) to study mitochondrial dysfunction related to ATP synthase deficiency. Heart mitochondria from LRPPRC KO mice were investigated and compared with WT mice. XL-MS was used to analyze proteome-wide protein-protein interactions, focusing on the mitochondrial respiratory system. Cross-links were quantified based on MS1 precursor intensities, and the differential abundance of the protein-protein interactions was assessed. Quantitative bottom-up proteomics analysis was also performed to infer changes in protein abundances.
Our preliminary data provide novel insights into ATP synthase deficiency as present in LS. Differences in cross-linked proteins between KO and WT mice were detected, revealing mutant-defected protein interaction partners relevant to this disease. Furthermore, differentially expressed proteins between KO and WT mitochondria indicate down-regulation of specific mt-DNA encoded proteins.

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