Mitochondrial Dysfunctions: Genetic and Cellular Implications Revealed by Various Model Organisms
Abstract
:1. Introduction
2. Model Organisms Used in Studies of the Cellular Role of Mitochondria
3. Mitochondrial Dysfunctions
4. Consequence of Mitochondrial Dysfunctions
4.1. Cellular Senescence and Aging Process
4.2. Mitochondrial Diseases
5. Conclusions
Author Contributions
Funding
Conflicts of Interest
References
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Organism | Description | Advantages of the Model Organism | Limitations of the Model Organism | References |
---|---|---|---|---|
Yeast Saccharomyces cerevisiae |
|
|
| [9,10,11,12] |
Nematode Caenorhabditis elegans |
|
|
| [13,14,15,16] |
Fruit fly Drosophila melanogaster |
|
|
| [17,18] |
Zebrafish Danio rerio |
|
|
| [19] |
Mouse Mus musculus |
|
|
| [20,21] |
Model Organism | Mitochondrial Aspect Related to the Aging Process | References |
---|---|---|
Yeast Saccharomyces cerevisiae |
| [75,76] |
Nematode Caenorhabditis elegans |
| [65,73,77] |
Fruit fly Drosophila melanogaster |
| [78,79,80,81] |
Zebrafish Danio rerio |
| [82,83] |
Mouse Mus musculus |
| [61,68,69,70] |
Disease | Genetic Change | Mitochondrial Dysfunctions | Sign and Symptoms of Disease | Model Organism for Studying Disease | References |
---|---|---|---|---|---|
Diseases caused by mutations in mtDNA | |||||
MELAS syndrome | MT-TL1 gene mutation |
| encephalopathy, lactic acidosis (due to increased glycolysis) | Saccharomyces cerevisiae, Danio rerio, Mus musculus | [86,87,88] |
MERRF syndrome | MT-TK gene mutation |
| progressive myoclonic epilepsy, muscle weakness, and muscle cells appear as frayed fibers | Danio rerio Mus musculus | [89,90] |
Leber’s hereditary optic neuropathy (LHON) | MT-ND1, MT-ND4, MT-ND4L, MT-ND6 genes mutation |
| loss of optic nerves due to degeneration of retinal ganglion cells and their axons | Danio rerio Mus musculus | [86,91,92] |
NARP syndrome | MT-ATP6 gene mutation |
| neuropathy, ataxia, retinitis pigmentosa | Saccharomyces cerevisiae | [93,94] |
Chronic progressive external ophthalmoplegia syndrome (CPEO) | deletions in mtDNA |
| myopathy, ptosis, retinitis pigmentosa, central nervous system dysfunction | Danio rerio | [91,95] |
Diseases caused by mutations in nuclear DNA | |||||
Charcot-Marie-Tooth disease type 2A (CMT2A) | MFN2 gene mutation |
| degeneration and loss of axons of myelin fibers in peripheral nerves | Saccharomyces cerevisiae, Danio rerio, Mus musculus | [96,97,98] |
Alpers-Huttenloch disease | POLG gene mutation |
| progressive loss of cognitive and motor skills | Caenorhabditis elegans, Danio rerio | [99,100] |
Friedreich’s ataxia | FXN gene mutation (encoding frataxin) |
| progressive ataxia of gait and limbs | Saccharomyces cerevisiae | [101,102] |
Leigh syndrome | mutations of genes encoding proteins of the respiratory chain complexes |
| necrotic changes in the basal ganglia, brainstem and midbrain, hypotonia, epilepsy, ataxia, lactic acidosis | Saccharomyces cerevisiae, Drosophila melanogaster, Danio rerio, Mus musculus | [86,91,103,104] |
Barth syndrome | TAZ gene mutation (encoding tafazzin) |
| decreased muscle tone, dilated cardiomyopathy, neutropenia | Saccharomyces cerevisiae, Drosophila melanogaster, Mus musculus | [105,106,107] |
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Stańczyk, M.; Szubart, N.; Maslanka, R.; Zadrag-Tecza, R. Mitochondrial Dysfunctions: Genetic and Cellular Implications Revealed by Various Model Organisms. Genes 2024, 15, 1153. https://doi.org/10.3390/genes15091153
Stańczyk M, Szubart N, Maslanka R, Zadrag-Tecza R. Mitochondrial Dysfunctions: Genetic and Cellular Implications Revealed by Various Model Organisms. Genes. 2024; 15(9):1153. https://doi.org/10.3390/genes15091153
Chicago/Turabian StyleStańczyk, Monika, Natalia Szubart, Roman Maslanka, and Renata Zadrag-Tecza. 2024. "Mitochondrial Dysfunctions: Genetic and Cellular Implications Revealed by Various Model Organisms" Genes 15, no. 9: 1153. https://doi.org/10.3390/genes15091153