Altered Gene Expression, Mitochondrial Damage and Oxidative Stress: Converging Routes in Motor Neuron Degeneration
Motor neuron diseases (MNDs) are a rather heterogeneous group of diseases, with either sporadic or genetic origin or both, all characterized by the progressive degeneration of motor neurons. At the cellular level, MNDs share features such as protein misfolding and aggregation, mitochondrial damage a...
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| Format: | Article |
| Language: | English |
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Wiley
2012-01-01
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| Series: | International Journal of Cell Biology |
| Online Access: | http://dx.doi.org/10.1155/2012/908724 |
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| _version_ | 1832560740625547264 |
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| author | Luisa Rossi Cristiana Valle Maria Teresa Carrì |
| author_facet | Luisa Rossi Cristiana Valle Maria Teresa Carrì |
| author_sort | Luisa Rossi |
| collection | DOAJ |
| description | Motor neuron diseases (MNDs) are a rather heterogeneous group of diseases, with either sporadic or genetic origin or both, all characterized by the progressive degeneration of motor neurons. At the cellular level, MNDs share features such as protein misfolding and aggregation, mitochondrial damage and energy deficit, and excitotoxicity and calcium mishandling. This is particularly well demonstrated in ALS, where both sporadic and familial forms share the same symptoms and pathological phenotype, with a prominent role for mitochondrial damage and resulting oxidative stress. Based on recent data, however, altered control of gene expression seems to be a most relevant, and previously overlooked, player in MNDs. Here we discuss which may be the links that make pathways apparently as different as altered gene expression, mitochondrial damage, and oxidative stress converge to generate a similar motoneuron-toxic phenotype. |
| format | Article |
| id | doaj-art-77166c86417748a586f412bcb4dae134 |
| institution | Kabale University |
| issn | 1687-8876 1687-8884 |
| language | English |
| publishDate | 2012-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | International Journal of Cell Biology |
| spelling | doaj-art-77166c86417748a586f412bcb4dae1342025-02-03T01:26:48ZengWileyInternational Journal of Cell Biology1687-88761687-88842012-01-01201210.1155/2012/908724908724Altered Gene Expression, Mitochondrial Damage and Oxidative Stress: Converging Routes in Motor Neuron DegenerationLuisa Rossi0Cristiana Valle1Maria Teresa Carrì2Department of Biology, University of Rome Tor Vergata, Via della Ricerca Scientifica, 00133 Rome, ItalyInstitute for Cell Biology and Neurobiology, CNR, 00100 Rome, ItalyDepartment of Biology, University of Rome Tor Vergata, Via della Ricerca Scientifica, 00133 Rome, ItalyMotor neuron diseases (MNDs) are a rather heterogeneous group of diseases, with either sporadic or genetic origin or both, all characterized by the progressive degeneration of motor neurons. At the cellular level, MNDs share features such as protein misfolding and aggregation, mitochondrial damage and energy deficit, and excitotoxicity and calcium mishandling. This is particularly well demonstrated in ALS, where both sporadic and familial forms share the same symptoms and pathological phenotype, with a prominent role for mitochondrial damage and resulting oxidative stress. Based on recent data, however, altered control of gene expression seems to be a most relevant, and previously overlooked, player in MNDs. Here we discuss which may be the links that make pathways apparently as different as altered gene expression, mitochondrial damage, and oxidative stress converge to generate a similar motoneuron-toxic phenotype.http://dx.doi.org/10.1155/2012/908724 |
| spellingShingle | Luisa Rossi Cristiana Valle Maria Teresa Carrì Altered Gene Expression, Mitochondrial Damage and Oxidative Stress: Converging Routes in Motor Neuron Degeneration International Journal of Cell Biology |
| title | Altered Gene Expression, Mitochondrial Damage and Oxidative Stress: Converging Routes in Motor Neuron Degeneration |
| title_full | Altered Gene Expression, Mitochondrial Damage and Oxidative Stress: Converging Routes in Motor Neuron Degeneration |
| title_fullStr | Altered Gene Expression, Mitochondrial Damage and Oxidative Stress: Converging Routes in Motor Neuron Degeneration |
| title_full_unstemmed | Altered Gene Expression, Mitochondrial Damage and Oxidative Stress: Converging Routes in Motor Neuron Degeneration |
| title_short | Altered Gene Expression, Mitochondrial Damage and Oxidative Stress: Converging Routes in Motor Neuron Degeneration |
| title_sort | altered gene expression mitochondrial damage and oxidative stress converging routes in motor neuron degeneration |
| url | http://dx.doi.org/10.1155/2012/908724 |
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