The 'mitochondrial contact site and cristae organising system' (MICOS) in health and human disease.

Autor: Eramo MJ; Department of Biochemistry and Molecular Biology, Monash Biomedicine Discovery Institute, 23 Innovation Walk, Monash University, 3800 Melbourne, Victoria, Australia., Lisnyak V; Department of Biochemistry and Molecular Biology, Monash Biomedicine Discovery Institute, 23 Innovation Walk, Monash University, 3800 Melbourne, Victoria, Australia., Formosa LE; Department of Biochemistry and Molecular Biology, Monash Biomedicine Discovery Institute, 23 Innovation Walk, Monash University, 3800 Melbourne, Victoria, Australia., Ryan MT; Department of Biochemistry and Molecular Biology, Monash Biomedicine Discovery Institute, 23 Innovation Walk, Monash University, 3800 Melbourne, Victoria, Australia.
Jazyk: angličtina
Zdroj: Journal of biochemistry [J Biochem] 2020 Mar 01; Vol. 167 (3), pp. 243-255.
DOI: 10.1093/jb/mvz111
Abstrakt: 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 Author(s) 2019. Published by Oxford University Press on behalf of the Japanese Biochemical Society. All rights reserved.)
Databáze: MEDLINE
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