Metabolic dysfunction-associated steatotic liver disease is a global health challenge driven by mitochondrial dysfunction, with structural and functional defects in mitochondrial cristae emerging as central contributors. This review synthesizes evidence linking the mitochondrial contact site and cristae organizing system complex dysfunction to the pathogenesis of metabolic dysfunction-associated steatotic liver disease. The mitochondrial contact site and cristae organizing system complex, spanning the folds of the inner mitochondrial membrane (i.e., cristae) and containing at least seven bona fide protein subunits in humans, whilst physically connecting with the sorting and assembly machinery complex to form the intermembrane space bridging supercomplex. Together, these complexes maintain cristae junctions and regulate broader mitochondrial quality control. However, despite the growing findings connecting mitochondrial reorganization to metabolic disorders, a crucial knowledge gap concerning the mechanistic integration of mitochondrial contact site and cristae organizing system subunits (e.g., Mic60 and Mic10 ) alterations with hepatic metabolic failure, inflammation, and fibrotic progression in metabolic dysfunction-associated steatotic liver disease remains unknown. Under metabolic stress, lipotoxicity, and aging, mitochondrial contact site and cristae organizing system subunits (e.g., Mic60 and Mic10 ) are downregulated, destabilizing cristae junctions and impairing oxidative phosphorylation. The review highlights the interplay between mitochondrial contact site and cristae organizing system instability and systemic metabolic collapse, emphasizing its role in driving hepatic inflammation and fibrotic progression. Emerging therapeutic strategies targeting mitochondrial contact site and cristae organizing system integrity, such as lysocardiolipin acyltransferase 1 inhibition, mitochondrial antioxidants such as MitoQ, and mitophagy enhancers such as urolithin A, are explored for their potential to restore cristae architecture and metabolic homeostasis. These approaches, alongside lifestyle interventions, may offer tailored solutions for aging populations with heightened organelle vulnerability. Understanding mitochondrial contact site and cristae organizing system dynamics provides a roadmap for precision therapies to halt the progression of metabolic dysfunction-associated steatotic liver disease.
Mweene et al. (Sat,) studied this question.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: