Impaired Autophagy and Defective Mitochondrial Function: Converging Paths on the Road to Motor Neuron Degeneration in ALS
Selective motor neuron degeneration is a hallmark of amyotrophic lateral sclerosis (ALS). Around 10 percent of all cases present as familial ALS (FALS), while sporadic ALS (SALS) accounts for the remaining 90 percent. Diverse genetic mutations leading to FALS have been identified, but the underlyin...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2016-03-01
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| Series: | Frontiers in Cellular Neuroscience |
| Subjects: | |
| Online Access: | http://journal.frontiersin.org/Journal/10.3389/fncel.2016.00044/full |
| _version_ | 1818133245031612416 |
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| author | Brittany M Edens Brittany M Edens Nimrod eMiller Nimrod eMiller Yong-Chao eMa Yong-Chao eMa |
| author_facet | Brittany M Edens Brittany M Edens Nimrod eMiller Nimrod eMiller Yong-Chao eMa Yong-Chao eMa |
| author_sort | Brittany M Edens |
| collection | DOAJ |
| description | Selective motor neuron degeneration is a hallmark of amyotrophic lateral sclerosis (ALS). Around 10 percent of all cases present as familial ALS (FALS), while sporadic ALS (SALS) accounts for the remaining 90 percent. Diverse genetic mutations leading to FALS have been identified, but the underlying causes of SALS remain largely unknown. Despite the heterogeneous and poorly understood etiology, different types of ALS exhibit overlapping pathology and common phenotypes, including protein aggregation and mitochondrial deficiencies. Here we review the current understanding of mechanisms leading to motor neuron degeneration in ALS, as they pertain to disrupted cellular clearance pathways, ATP biogenesis calcium buffering and mitochondrial dynamics. Through focusing on impaired autophagic and mitochondrial functions, we highlight how the convergence of diverse cellular processes and pathways contributes to common pathology in motor neuron degeneration. |
| first_indexed | 2024-12-11T08:49:39Z |
| format | Article |
| id | doaj.art-8efddb32c9394f159f331cb250133c21 |
| institution | Directory Open Access Journal |
| issn | 1662-5102 |
| language | English |
| last_indexed | 2024-12-11T08:49:39Z |
| publishDate | 2016-03-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Cellular Neuroscience |
| spelling | doaj.art-8efddb32c9394f159f331cb250133c212022-12-22T01:14:03ZengFrontiers Media S.A.Frontiers in Cellular Neuroscience1662-51022016-03-011010.3389/fncel.2016.00044177796Impaired Autophagy and Defective Mitochondrial Function: Converging Paths on the Road to Motor Neuron Degeneration in ALSBrittany M Edens0Brittany M Edens1Nimrod eMiller2Nimrod eMiller3Yong-Chao eMa4Yong-Chao eMa5Northwestern University Feinberg School of MedicineLurie Children's Hospital of ChicagoNorthwestern University Feinberg School of MedicineLurie Children's Hospital of ChicagoNorthwestern University Feinberg School of MedicineLurie Children's Hospital of ChicagoSelective motor neuron degeneration is a hallmark of amyotrophic lateral sclerosis (ALS). Around 10 percent of all cases present as familial ALS (FALS), while sporadic ALS (SALS) accounts for the remaining 90 percent. Diverse genetic mutations leading to FALS have been identified, but the underlying causes of SALS remain largely unknown. Despite the heterogeneous and poorly understood etiology, different types of ALS exhibit overlapping pathology and common phenotypes, including protein aggregation and mitochondrial deficiencies. Here we review the current understanding of mechanisms leading to motor neuron degeneration in ALS, as they pertain to disrupted cellular clearance pathways, ATP biogenesis calcium buffering and mitochondrial dynamics. Through focusing on impaired autophagic and mitochondrial functions, we highlight how the convergence of diverse cellular processes and pathways contributes to common pathology in motor neuron degeneration.http://journal.frontiersin.org/Journal/10.3389/fncel.2016.00044/fullAutophagyMitochondriaMotor Neuron DiseaseOxidative StressALScalcium homeostasis |
| spellingShingle | Brittany M Edens Brittany M Edens Nimrod eMiller Nimrod eMiller Yong-Chao eMa Yong-Chao eMa Impaired Autophagy and Defective Mitochondrial Function: Converging Paths on the Road to Motor Neuron Degeneration in ALS Frontiers in Cellular Neuroscience Autophagy Mitochondria Motor Neuron Disease Oxidative Stress ALS calcium homeostasis |
| title | Impaired Autophagy and Defective Mitochondrial Function: Converging Paths on the Road to Motor Neuron Degeneration in ALS |
| title_full | Impaired Autophagy and Defective Mitochondrial Function: Converging Paths on the Road to Motor Neuron Degeneration in ALS |
| title_fullStr | Impaired Autophagy and Defective Mitochondrial Function: Converging Paths on the Road to Motor Neuron Degeneration in ALS |
| title_full_unstemmed | Impaired Autophagy and Defective Mitochondrial Function: Converging Paths on the Road to Motor Neuron Degeneration in ALS |
| title_short | Impaired Autophagy and Defective Mitochondrial Function: Converging Paths on the Road to Motor Neuron Degeneration in ALS |
| title_sort | impaired autophagy and defective mitochondrial function converging paths on the road to motor neuron degeneration in als |
| topic | Autophagy Mitochondria Motor Neuron Disease Oxidative Stress ALS calcium homeostasis |
| url | http://journal.frontiersin.org/Journal/10.3389/fncel.2016.00044/full |
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