Genetic Neuropathy Due to Impairments in Mitochondrial Dynamics
Mitochondria are dynamic organelles capable of fusing, dividing, and moving about the cell. These properties are especially important in neurons, which in addition to high energy demand, have unique morphological properties with long axons. Notably, mitochondrial dysfunction causes a variety of neur...
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| Format: | Article |
| Language: | English |
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MDPI AG
2021-03-01
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| Series: | Biology |
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| Online Access: | https://www.mdpi.com/2079-7737/10/4/268 |
| _version_ | 1797539889866080256 |
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| author | Govinda Sharma Gerald Pfeffer Timothy E. Shutt |
| author_facet | Govinda Sharma Gerald Pfeffer Timothy E. Shutt |
| author_sort | Govinda Sharma |
| collection | DOAJ |
| description | Mitochondria are dynamic organelles capable of fusing, dividing, and moving about the cell. These properties are especially important in neurons, which in addition to high energy demand, have unique morphological properties with long axons. Notably, mitochondrial dysfunction causes a variety of neurological disorders including peripheral neuropathy, which is linked to impaired mitochondrial dynamics. Nonetheless, exactly why peripheral neurons are especially sensitive to impaired mitochondrial dynamics remains somewhat enigmatic. Although the prevailing view is that longer peripheral nerves are more sensitive to the loss of mitochondrial motility, this explanation is insufficient. Here, we review pathogenic variants in proteins mediating mitochondrial fusion, fission and transport that cause peripheral neuropathy. In addition to highlighting other dynamic processes that are impacted in peripheral neuropathies, we focus on impaired mitochondrial quality control as a potential unifying theme for why mitochondrial dysfunction and impairments in mitochondrial dynamics in particular cause peripheral neuropathy. |
| first_indexed | 2024-03-10T12:52:14Z |
| format | Article |
| id | doaj.art-61dab7cef0c2455d8cc947a24fab8e9e |
| institution | Directory Open Access Journal |
| issn | 2079-7737 |
| language | English |
| last_indexed | 2024-03-10T12:52:14Z |
| publishDate | 2021-03-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Biology |
| spelling | doaj.art-61dab7cef0c2455d8cc947a24fab8e9e2023-11-21T12:58:54ZengMDPI AGBiology2079-77372021-03-0110426810.3390/biology10040268Genetic Neuropathy Due to Impairments in Mitochondrial DynamicsGovinda Sharma0Gerald Pfeffer1Timothy E. Shutt2Departments of Medical Genetics and Biochemistry & Molecular Biology, Cumming School of Medicine, Alberta Children’s Hospital Research Institute, Hotchkiss Brain Institute, University of Calgary, Calgary, AB T2N 4N1, CanadaDepartments of Clinical Neurosciences and Medical Genetics, Cumming School of Medicine, Hotchkiss Brain Institute, Alberta Child Health Research Institute, University of Calgary, Calgary, AB T2N 4N1, CanadaDepartments of Medical Genetics and Biochemistry & Molecular Biology, Cumming School of Medicine, Alberta Children’s Hospital Research Institute, Hotchkiss Brain Institute, University of Calgary, Calgary, AB T2N 4N1, CanadaMitochondria are dynamic organelles capable of fusing, dividing, and moving about the cell. These properties are especially important in neurons, which in addition to high energy demand, have unique morphological properties with long axons. Notably, mitochondrial dysfunction causes a variety of neurological disorders including peripheral neuropathy, which is linked to impaired mitochondrial dynamics. Nonetheless, exactly why peripheral neurons are especially sensitive to impaired mitochondrial dynamics remains somewhat enigmatic. Although the prevailing view is that longer peripheral nerves are more sensitive to the loss of mitochondrial motility, this explanation is insufficient. Here, we review pathogenic variants in proteins mediating mitochondrial fusion, fission and transport that cause peripheral neuropathy. In addition to highlighting other dynamic processes that are impacted in peripheral neuropathies, we focus on impaired mitochondrial quality control as a potential unifying theme for why mitochondrial dysfunction and impairments in mitochondrial dynamics in particular cause peripheral neuropathy.https://www.mdpi.com/2079-7737/10/4/268neuropathymitochondriamitochondrial dynamicsfusionfissiontransport |
| spellingShingle | Govinda Sharma Gerald Pfeffer Timothy E. Shutt Genetic Neuropathy Due to Impairments in Mitochondrial Dynamics Biology neuropathy mitochondria mitochondrial dynamics fusion fission transport |
| title | Genetic Neuropathy Due to Impairments in Mitochondrial Dynamics |
| title_full | Genetic Neuropathy Due to Impairments in Mitochondrial Dynamics |
| title_fullStr | Genetic Neuropathy Due to Impairments in Mitochondrial Dynamics |
| title_full_unstemmed | Genetic Neuropathy Due to Impairments in Mitochondrial Dynamics |
| title_short | Genetic Neuropathy Due to Impairments in Mitochondrial Dynamics |
| title_sort | genetic neuropathy due to impairments in mitochondrial dynamics |
| topic | neuropathy mitochondria mitochondrial dynamics fusion fission transport |
| url | https://www.mdpi.com/2079-7737/10/4/268 |
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