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- Title
The 'mitochondrial contact site and cristae organising system' (MICOS) in health and human disease.
- Authors
Eramo, Matthew J; Lisnyak, Valerie; Formosa, Luke E; Ryan, Michael T
- Abstract
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.
- Subjects
MUSCULAR dystrophy; NEURODEGENERATION; DISEASES; DIABETES; MUSCLE diseases
- Publication
Journal of Biochemistry, 2020, Vol 167, Issue 3, p243
- ISSN
0021-924X
- Publication type
Article
- DOI
10.1093/jb/mvz111