Comprehensive Analysis of Mitochondrial Dynamics Alterations in Heart Diseases

Morciano, Giampaolo; Boncompagni, Caterina; Ramaccini, Daniela; Pedriali, Gaia; Bouhamida, Esmaa; Tremoli, Elena; Giorgi, Carlotta; Pinton, Paolo

doi:10.3390/ijms24043414

Cited by 14 publications

(4 citation statements)

References 189 publications

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“…The disruption of the normal mitochondrial fusion process has been shown to lead to smaller fragmented mitochondria, causing impaired cellular respiration [ 16 , 34 ]. In addition, it has been associated with a variety of neurodegenerative and cardiovascular diseases [ 35 , 36 ]. The analysis of MD-associated proteins in the cardiac tissue of CD mice revealed a shift toward fission, similar to that observed in a variety of cardiovascular diseases [ 37 ].…”

Section: Discussionmentioning

confidence: 99%

Dysfunctional Mitochondria in the Cardiac Fibers of a Williams–Beuren Syndrome Mouse Model

Abdalla

Tobías-Baraja

González

et al. 2023

IJMS

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Williams–Beuren syndrome (WBS) is a rare neurodevelopmental disorder that, together with a rather characteristic neurocognitive profile, presents a strong cardiovascular phenotype. The cardiovascular features of WBS are mainly related to a gene dosage effect due to hemizygosity of the elastin (ELN) gene; however, the phenotypic variability between WBS patients indicates the presence of important modulators of the clinical impact of elastin deficiency. Recently, two genes within the WBS region have been linked to mitochondrial dysfunction. Numerous cardiovascular diseases are related to mitochondrial dysfunction; therefore, it could be a modulator of the phenotype present in WBS. Here, we analyze mitochondrial function and dynamics in cardiac tissue from a WBS complete deletion (CD) model. Our research reveals that cardiac fiber mitochondria from CD animals have altered mitochondrial dynamics, accompanied by respiratory chain dysfunction with decreased ATP production, reproducing alterations observed in fibroblasts from WBS patients. Our results highlight two major factors: on the one hand, that mitochondrial dysfunction is probably a relevant mechanism underlying several risk factors associated with WBS disease; on the other, the CD murine model mimics the mitochondrial phenotype of WBS and could be a great model for carrying out preclinical tests on drugs targeting the mitochondria.

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Section: Discussionmentioning

confidence: 99%

Dysfunctional Mitochondria in the Cardiac Fibers of a Williams–Beuren Syndrome Mouse Model

Abdalla

Tobías-Baraja

González

et al. 2023

IJMS

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“…Mitochondria play a major role in cell function by generating ATP through oxidative phosphorylation of fatty acids. They adapt to the needs of the cell and the extracellular space by forming a network of mitochondria that change in number and appearance [67][68][69].…”

Section: Mechanisms Of Mitochondrial Dysfunction In Cardiorenal Syndr...mentioning

confidence: 99%

The Role Of Oxidative Stress As A Mechanism In The Pathogenesis Of Heart Failure In Acute Kidney Injury

Tasic

2024

Preprint

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Despite a large amount of research on synchronous and mutually induced kidney and heart damage, the basis of the disease is still not fully clarified. Healthy mitochondria are essential for normal kidney and heart function. Mitochondrial dysfunction occurs when the clearance or process of generation and fragmentation of mitochondria is disturbed. The kidney is the second organ after the heart in the number of mitochondria. Kidney tubules are rich in mitochondria due to the high energy requirements for absorption processes of large amounts of ultrafiltrate and dissolved substances. The place of action of oxidative stress is the influence on the balance in the production and breakdown of mitochondrial reactive oxygen species . A more precise determination of the place and role of key factors that play a role in the onset of the disease is necessary for understanding the nature of the onset of the disease and the creation of therapy in the future. The narrative review integrates results found in previously performed studies which have evaluated oxidative stress participation in renocardial syndrome type 3.

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“…Importantly, the reversible nature of DRP1 phosphorylation profoundly influences its functions [85]. For instance, calcineurin-mediated dephosphorylation of p-DRP1-S637 recruits DRP1 to mitochondria, promoting mitochondrial fragmentation and synaptic dysfunction [86].…”

Section: Protein Machinery In Mitochondrial Fissionmentioning

confidence: 99%

Mitochondrial Dynamics in Neurodegenerative Diseases: Unraveling the Role of Fusion and Fission Processes

Grel,

Woznica,

Ratajczak

et al. 2023

IJMS

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Neurodegenerative diseases (NDs) are a diverse group of disorders characterized by the progressive degeneration and death of neurons, leading to a range of neurological symptoms. Despite the heterogeneity of these conditions, a common denominator is the implication of mitochondrial dysfunction in their pathogenesis. Mitochondria play a crucial role in creating biomolecules, providing energy through adenosine triphosphate (ATP) generated by oxidative phosphorylation (OXPHOS), and producing reactive oxygen species (ROS). When they’re not functioning correctly, becoming fragmented and losing their membrane potential, they contribute to these diseases. In this review, we explore how mitochondria fuse and undergo fission, especially in the context of NDs. We discuss the genetic and protein mutations linked to these diseases and how they impact mitochondrial dynamics. We also look at the key regulatory proteins in fusion (MFN1, MFN2, and OPA1) and fission (DRP1 and FIS1), including their post-translational modifications. Furthermore, we highlight potential drugs that can influence mitochondrial dynamics. By unpacking these complex processes, we aim to direct research towards treatments that can improve life quality for people with these challenging conditions.

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Comprehensive Analysis of Mitochondrial Dynamics Alterations in Heart Diseases

Cited by 14 publications

References 189 publications

Dysfunctional Mitochondria in the Cardiac Fibers of a Williams–Beuren Syndrome Mouse Model

Dysfunctional Mitochondria in the Cardiac Fibers of a Williams–Beuren Syndrome Mouse Model

The Role Of Oxidative Stress As A Mechanism In The Pathogenesis Of Heart Failure In Acute Kidney Injury

Mitochondrial Dynamics in Neurodegenerative Diseases: Unraveling the Role of Fusion and Fission Processes

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