Abstract
Mitochondrial adaptation to different physiological conditions highly relies on the regulation of mitochondrial ultrastructure, particularly at the level of cristae compartment. Cristae represent the membrane hub where most of the respiratory complexes embed to account for OXPHOS and energy production in the form of adenosine triphosphate (ATP). Changes in cristae number and shape define the respiratory capacity as well as cell viability. The identification of key regulators of cristae morphology and the understanding of their contribution to the mitochondrial ultrastructure and function have become an strategic goal to understand mitochondrial disorders and to exploit as therapeutic targets. This review summarizes the known regulators of cristae ultrastructure and discusses their contribution and implications for mitochondrial dysfunction.
Keywords:
ATP synthase dimers; Cristae ultrastructure; MICOS; Mitochondria; OPA1.
Copyright © 2017 Elsevier Inc. All rights reserved.
Publication types
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Research Support, Non-U.S. Gov't
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Review
MeSH terms
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Adenosine Triphosphate / biosynthesis
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Cell Survival
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Electron Transport Chain Complex Proteins / genetics
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Electron Transport Chain Complex Proteins / metabolism*
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Eukaryotic Cells / metabolism*
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Eukaryotic Cells / ultrastructure
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GTP Phosphohydrolases / genetics
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GTP Phosphohydrolases / metabolism
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Gene Expression Regulation
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Humans
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Mitochondria / metabolism*
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Mitochondria / ultrastructure
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Mitochondrial Membrane Transport Proteins / genetics
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Mitochondrial Membrane Transport Proteins / metabolism*
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Mitochondrial Membranes / metabolism*
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Mitochondrial Membranes / ultrastructure
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Mitochondrial Proton-Translocating ATPases / genetics
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Mitochondrial Proton-Translocating ATPases / metabolism*
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Organelle Shape / physiology
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Oxidative Phosphorylation
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Protein Multimerization
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Signal Transduction
Substances
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Electron Transport Chain Complex Proteins
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Mitochondrial Membrane Transport Proteins
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Adenosine Triphosphate
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GTP Phosphohydrolases
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OPA1 protein, human
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Mitochondrial Proton-Translocating ATPases