Mitochondrial Fission and Fusion: Molecular Mechanisms, Biological Functions, and Related Disorders

Mitochondria are dynamic organelles that undergo fusion and fission. These active processes occur continuously and simultaneously and are mediated by nuclear-DNA-encoded proteins that act on mitochondrial membranes. The balance between fusion and fission determines the mitochondrial morphology and a...

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Main Authors: Mode Al Ojaimi, Azza Salah, Ayman W. El-Hattab
Format: Article
Language:English
Published: MDPI AG 2022-09-01
Series:Membranes
Subjects:
Online Access:https://www.mdpi.com/2077-0375/12/9/893
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author Mode Al Ojaimi
Azza Salah
Ayman W. El-Hattab
author_facet Mode Al Ojaimi
Azza Salah
Ayman W. El-Hattab
author_sort Mode Al Ojaimi
collection DOAJ
description Mitochondria are dynamic organelles that undergo fusion and fission. These active processes occur continuously and simultaneously and are mediated by nuclear-DNA-encoded proteins that act on mitochondrial membranes. The balance between fusion and fission determines the mitochondrial morphology and adapts it to the metabolic needs of the cells. Therefore, these two processes are crucial to optimize mitochondrial function and its bioenergetics abilities. Defects in mitochondrial proteins involved in fission and fusion due to pathogenic variants in the genes encoding them result in disruption of the equilibrium between fission and fusion, leading to a group of mitochondrial diseases termed disorders of mitochondrial dynamics. In this review, the molecular mechanisms and biological functions of mitochondrial fusion and fission are first discussed. Then, mitochondrial disorders caused by defects in fission and fusion are summarized, including disorders related to <i>MFN2, MSTO1, OPA1, YME1L1, FBXL4, DNM1L</i>, and <i>MFF</i> genes.
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spelling doaj.art-291d1e9e976e44b789fdf2f2ff610bbd2023-11-23T17:43:10ZengMDPI AGMembranes2077-03752022-09-0112989310.3390/membranes12090893Mitochondrial Fission and Fusion: Molecular Mechanisms, Biological Functions, and Related DisordersMode Al Ojaimi0Azza Salah1Ayman W. El-Hattab2College of Medicine, University of Sharjah, Sharjah 27272, United Arab EmiratesPediatrics Department, University Hospital Sharjah, Sharjah 72772, United Arab EmiratesCollege of Medicine, University of Sharjah, Sharjah 27272, United Arab EmiratesMitochondria are dynamic organelles that undergo fusion and fission. These active processes occur continuously and simultaneously and are mediated by nuclear-DNA-encoded proteins that act on mitochondrial membranes. The balance between fusion and fission determines the mitochondrial morphology and adapts it to the metabolic needs of the cells. Therefore, these two processes are crucial to optimize mitochondrial function and its bioenergetics abilities. Defects in mitochondrial proteins involved in fission and fusion due to pathogenic variants in the genes encoding them result in disruption of the equilibrium between fission and fusion, leading to a group of mitochondrial diseases termed disorders of mitochondrial dynamics. In this review, the molecular mechanisms and biological functions of mitochondrial fusion and fission are first discussed. Then, mitochondrial disorders caused by defects in fission and fusion are summarized, including disorders related to <i>MFN2, MSTO1, OPA1, YME1L1, FBXL4, DNM1L</i>, and <i>MFF</i> genes.https://www.mdpi.com/2077-0375/12/9/893mitochondrial diseasesmitochondrial fissionmitochondrial fusionmitochondrial dynamics
spellingShingle Mode Al Ojaimi
Azza Salah
Ayman W. El-Hattab
Mitochondrial Fission and Fusion: Molecular Mechanisms, Biological Functions, and Related Disorders
Membranes
mitochondrial diseases
mitochondrial fission
mitochondrial fusion
mitochondrial dynamics
title Mitochondrial Fission and Fusion: Molecular Mechanisms, Biological Functions, and Related Disorders
title_full Mitochondrial Fission and Fusion: Molecular Mechanisms, Biological Functions, and Related Disorders
title_fullStr Mitochondrial Fission and Fusion: Molecular Mechanisms, Biological Functions, and Related Disorders
title_full_unstemmed Mitochondrial Fission and Fusion: Molecular Mechanisms, Biological Functions, and Related Disorders
title_short Mitochondrial Fission and Fusion: Molecular Mechanisms, Biological Functions, and Related Disorders
title_sort mitochondrial fission and fusion molecular mechanisms biological functions and related disorders
topic mitochondrial diseases
mitochondrial fission
mitochondrial fusion
mitochondrial dynamics
url https://www.mdpi.com/2077-0375/12/9/893
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AT azzasalah mitochondrialfissionandfusionmolecularmechanismsbiologicalfunctionsandrelateddisorders
AT aymanwelhattab mitochondrialfissionandfusionmolecularmechanismsbiologicalfunctionsandrelateddisorders