Abstract
The mitochondrial network is a dynamic organization within eukaryotic cells that participates in a variety of essential cellular processes, such as adenosine triphosphate (ATP) synthesis, central metabolism, apoptosis and inflammation. The mitochondrial network is balanced between rates of fusion and fission that respond to pathophysiologic signals to coordinate appropriate mitochondrial processes. Mitochondrial fusion and fission are regulated by proteins that either reside in or translocate to the inner or outer mitochondrial membranes or are soluble in the inter-membrane space. Mitochondrial fission and fusion are performed by guanosine triphosphatases (GTPases) on the outer and inner mitochondrial membranes with the assistance of other mitochondrial proteins. Due to the essential nature of mitochondrial function for cellular homeostasis, regulation of mitochondrial dynamics is under strict control. Some of the mechanisms used to regulate the function of these proteins are post-translational proteolysis and/or turnover, and this review will discuss these mechanisms required for correct mitochondrial network organization.
Highlights
Mitochondria are the power houses of eukaryotic cells, generating chemical energy in the form of adenosine triphosphate (ATP) by the oxidative phosphorylation (OXPHOS) system
Inner mitochondrial membrane (IMM) fusion is mediated by optic atrophy 1 (OPA1), a dynamin-related guanosine triphosphatases (GTPases) protein, which is associated with different functions such as maintenance of the respiratory chain, IMM potential, mtDNA and control of apoptosis [8]
Constitutive proteolytic processing by YME1L and/or OMA1 generates a balance of short and long isoforms that are released into the intermembrane space (IMS) or tethered to the IMM respectively
Summary
Mitochondria are the power houses of eukaryotic cells, generating chemical energy in the form of adenosine triphosphate (ATP) by the oxidative phosphorylation (OXPHOS) system. Inner mitochondrial membrane (IMM) fusion is mediated by OPA1, a dynamin-related GTPase protein, which is associated with different functions such as maintenance of the respiratory chain, IMM potential, mtDNA and control of apoptosis [8]. A subgroup of Optic Atrophy is caused by mutations in the mitochondrial AAA-ATPase protease YME1L [29] Another disease resulting from defective mitochondrial dynamics are the Encephalopathies due to defective Mitochondrial and Peroxisomal Fission (EMPF). Fragmentation causes the accumulation of calcium in mitochondria which is a common activator of the mitochondrial permeability transition, in the case of pro-fission proteins such as DRP1 and Fis1 [39]. This review will focus on these two types of post-translational regulation of mitochondrial dynamics proteins
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