The 'mitochondrial contact site and cristae organising system' (MICOS) in health and human disease
| Autor: | Matthew J Eramo, Luke E. Formosa, Michael T. Ryan, Valerie Lisnyak |
|---|---|
| Rok vydání: | 2019 |
| Předmět: |
Mitochondrion
Biology Biochemistry Mitochondrial Proteins 03 medical and health sciences 0302 clinical medicine Human disease Membrane organization Alzheimer Disease Neoplasms Diabetes Mellitus Humans Disease process Molecular Biology 030304 developmental biology 0303 health sciences Parkinson Disease General Medicine Cell biology Mitochondria Mitochondrial abnormalities Cardiovascular Diseases Mitochondrial Membranes Mitochondrial cristae Nervous System Diseases 030217 neurology & neurosurgery |
| Zdroj: | Journal of biochemistry. 167(3) |
| ISSN: | 1756-2651 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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