The ‘mitochondrial contact site and cristae organising system’ (MICOS) is an essential protein complex that promotes the formation, maintenance and stability of mitochondrial cristae. As such, loss of core MICOS components disrupts cristae structure and impairs mitochondrial function. Aberrant mitochondrial cristae morphology and diminished mitochondrial function is a pathological hallmark observed across many human diseases such as neurodegenerative conditions, obesity and diabetes mellitus, cardiomyopathy, and in muscular dystrophies and myopathies. While mitochondrial abnormalities are often an associated secondary effect to the pathological disease process, a direct role for the MICOS in health and human disease is emerging. This review describes the role of MICOS in the maintenance of mitochondrial architecture and summarizes both the direct and associated roles of the MICOS in human disease.

The role of the MICOS in the maintenance of mitochondrial health and human disease is only just emerging. Maintenance of the MICOS is essential for normal mitochondrial and cellular function. However, how the cristae abnormalities and mitochondrial dysfunction observed in many human diseases are influenced by changes to MICOS subunits remains unclear. Further research utilising in vivo animal models is required to understand the direct contributions of MICOS to shaping tissue-specific mitochondrial cristae architecture, and how loss of these subunits can initiate, promote or contribute to the progression of disease. In addition, further structural and functional characterisation of the MICOS will continue to uncover novel MICOS interactors, potentially with their own links to human disease.

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