Mitochondrial Dynamics in Mitochondrial Diseases

Suárez-Rivero, Juan M.; Paz, Marina Villanueva; Cruz‐Ojeda, Patricia de la; Mata, Mario de la; Cotán, David; Oropesa-Ávila, Manuel; Lavera, Isabel de; Álvarez-Córdoba, Mónica; Luzón-Hidalgo, Raquel; Sánchez-Alcázar, José A.

doi:10.3390/diseases5010001

Cited by 149 publications

(106 citation statements)

References 112 publications

(134 reference statements)

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“…The molecular machinery that mediates this organelle fission and fusion is thus necessary to maintain MT integrity and is tightly regulated by post-translational modifications and protein-protein interactions [2].…”

Section: Introductionmentioning

confidence: 99%

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Mitochondrial Dynamics in Basal and Stressful Conditions

Zemirli

Morel

Molino

2018

IJMS

131

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The historical role of mitochondria resides in converting the energy released during the oxidation of macromolecules (carbohydrates, lipids and proteins) into adenosine tri-phosphate, a major form of chemically stored energy which sustains cell growth and homeostasis. Beyond this role in bioenergetics regulation, mitochondria play a role in several other cellular processes including lipid metabolism, cellular calcium homeostasis, autophagy and immune responses. Furthermore, mitochondria are highly dynamic organelles: as all other cellular endomembranes, they are continuously moving along cytoskeleton, and, most importantly, they constantly interact one with each other by membrane tethering, fusion and fission. This review aims to highlight the tight correlation between the morphodynamics of mitochondria and their biological function(s), in physiological as well as stress conditions, in particular nutrient deprivation, pathogen attack and some human diseases. Finally, we emphasize some crosstalk between the fusion/fission machinery and the autophagy pathway to ending on some speculative hypothesis to inspire future research in the field.

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Section: Introductionmentioning

confidence: 99%

“…A defective mitochondrial dynamic machinery could have important physiological consequences including embryonic lethality and diseases [2] (Table 1), suggesting that fission and fusion go far beyond the morphological aspect of mitochondria. Mitochondrial dynamics have been linked to multiple mitochondrial functions.…”

Section: Introductionmentioning

confidence: 99%

Mitochondrial Dynamics in Basal and Stressful Conditions

Zemirli

Morel

Molino

2018

IJMS

131

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“…Under normal circumstances, fusion and fission occur simultaneously and at approximately the same rate; accordingly, any change in the homeostatic balance due to cellular stimuli may result in elongated or fragmented mitochondria. The physiological significance of mitochondrial fusion and/or fission dysfunction is illustrated by the growing number of pathological conditions related directly to mitochondrial homeostasis (Table ) …”

Section: Mitochondrial Homeostasismentioning

confidence: 99%

“…The physiological significance of mitochondrial fusion and/or fission dysfunction is illustrated by the growing number of pathological conditions related directly to mitochondrial homeostasis (Table 1). 17,18…”

Section: Under Normal Circumstances Fusion and Fission Occur Simultamentioning

confidence: 99%

Tying trafficking to fusion and fission at the mighty mitochondria

Farmer

Naslavsky

Caplan

2018

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The mitochondrion is a unique organelle that serves as the main site of ATP generation needed for energy in the cell. However, mitochondria also play essential roles in cell death through apoptosis and necrosis, as well as a variety of crucial functions related to stress regulation, autophagy, lipid synthesis and calcium storage. There is a growing appreciation that mitochondrial function is regulated by the dynamics of its membrane fusion and fission; longer, fused mitochondria are optimal for ATP generation, whereas fission of mitochondria facilitates mitophagy and cell division. Despite the significance of mitochondrial homeostasis for such crucial cellular events, the intricate regulation of mitochondrial fusion and fission is only partially understood. Until very recently, only a single mitochondrial fission protein had been identified. Moreover, only now have researchers turned to address the upstream machinery that regulates mitochondrial fusion and fission proteins. Herein, we review the known GTPases involved in mitochondrial fusion and fission, but also highlight recent studies that address the mechanisms by which these GTPases are regulated. In particular, we draw attention to a substantial new body of literature linking endocytic regulatory proteins, such as the retromer VPS35 cargo selection complex subunit, to mitochondrial homeostasis. These recent studies suggest that relationships and cross-regulation between endocytic and mitochondrial pathways may be more widespread than previously assumed.

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“…Progress in mitochondrial research has shown that mitochondria are versatile and dynamic organelles that can undergo fusion, fission, biogenesis and autophagic elimination to maintain mitochondrial network quality control in response to various cellular signals (Suárez‐Rivero et al, ). One important breakthrough in the field of Mitochondrial Research was the delineation of how mitochondrial dynamics is linked to bioenergetics and signaling functions of the organelle.…”

Section: Mitomeeting Opening Lectures: “Leopoldo Demeis Conference”mentioning

confidence: 99%