» Articles » PMID: 36135912

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

Overview
Date 2022 Sep 22
PMID 36135912
Authors
Affiliations
Soon will be listed here.
Abstract

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 , and genes.

Citing Articles

Mitochondria-associated endoplasmic reticulum membranes and myocardial ischemia: from molecular mechanisms to therapeutic strategies.

Chen C, Dai G, Fan M, Wang X, Niu K, Gao W J Transl Med. 2025; 23(1):277.

PMID: 40050915 PMC: 11884070. DOI: 10.1186/s12967-025-06262-3.


Visual Analysis of Research Hotspots and Trends on Mitochondria-Associated Membranes in the Past 20 Years-Focused on Neurodegenerative Diseases.

Du Y, Duan C, Zhu X, Lyu M, Chen J, Wei Y Mol Neurobiol. 2025; .

PMID: 39964584 DOI: 10.1007/s12035-025-04722-x.


DRP1: shedding light on the complex nexus of mitochondrial fission and breast cancer.

You L, Wang M, Liu X, Song M, Zhou J, Feng J Future Oncol. 2025; 21(5):593-603.

PMID: 39936355 PMC: 11845117. DOI: 10.1080/14796694.2024.2447813.


Mitochondrial pathways of copper neurotoxicity: focus on mitochondrial dynamics and mitophagy.

Aschner M, Skalny A, Lu R, Martins A, Tizabi Y, Nekhoroshev S Front Mol Neurosci. 2024; 17:1504802.

PMID: 39703721 PMC: 11655512. DOI: 10.3389/fnmol.2024.1504802.


SMN Deficiency Induces an Early Non-Atrophic Myopathy with Alterations in the Contractile and Excitatory Coupling Machinery of Skeletal Myofibers in the SMN∆7 Mouse Model of Spinal Muscular Atrophy.

Berciano M, Gatius A, Puente-Bedia A, Rufino-Gomez A, Tarabal O, Rodriguez-Rey J Int J Mol Sci. 2024; 25(22).

PMID: 39596480 PMC: 11595111. DOI: 10.3390/ijms252212415.


References
1.
Gegg M, Cooper J, Chau K, Rojo M, Schapira A, Taanman J . Mitofusin 1 and mitofusin 2 are ubiquitinated in a PINK1/parkin-dependent manner upon induction of mitophagy. Hum Mol Genet. 2010; 19(24):4861-70. PMC: 3583518. DOI: 10.1093/hmg/ddq419. View

2.
McCormick E, Zolkipli-Cunningham Z, Falk M . Mitochondrial disease genetics update: recent insights into the molecular diagnosis and expanding phenotype of primary mitochondrial disease. Curr Opin Pediatr. 2018; 30(6):714-724. PMC: 6467265. DOI: 10.1097/MOP.0000000000000686. View

3.
Bonnen P, Yarham J, Besse A, Wu P, Faqeih E, Al-Asmari A . Mutations in FBXL4 cause mitochondrial encephalopathy and a disorder of mitochondrial DNA maintenance. Am J Hum Genet. 2013; 93(3):471-81. PMC: 3769921. DOI: 10.1016/j.ajhg.2013.07.017. View

4.
Ardicli D, Sarkozy A, Zaharieva I, Deshpande C, Bodi I, Siddiqui A . A novel case of MSTO1 gene related congenital muscular dystrophy with progressive neurological involvement. Neuromuscul Disord. 2019; 29(6):448-455. DOI: 10.1016/j.nmd.2019.03.011. View

5.
van der Bliek A, Shen Q, Kawajiri S . Mechanisms of mitochondrial fission and fusion. Cold Spring Harb Perspect Biol. 2013; 5(6). PMC: 3660830. DOI: 10.1101/cshperspect.a011072. View