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Death Receptors in the Selective Degeneration of Motoneurons in Amyotrophic Lateral Sclerosis

Death Receptors in the Selective Degeneration of Motoneurons in Amyotrophic Lateral Sclerosis While studies on death receptors have long been restricted to immune cells, the last decade has provided a strong body of evidence for their implication in neuronal death and hence neurodegenerative disorders such as amyotrophic lateral sclerosis (ALS). ALS is a fatal paralytic disorder that primarily affects motoneurons in the brain and spinal cord. A neuroinflammatory process, associated with astrocyte and microglial activation as well as infiltration of immune cells, accompanies motoneuron degeneration and supports the contribution of non-cell-autonomous mechanisms in the disease. Hallmarks of Fas, TNFR, LT-βR, and p7 signaling have been observed in both animal models and ALS patients. This review summarizes to date knowledge of the role of death receptors in ALS and the link existing between the selective loss of motoneurons and neuroinflammation. It further suggests how this recent evidence could be included in an ultimate multiapproach to treat patients. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Neurodegenerative Diseases Hindawi Publishing Corporation

Death Receptors in the Selective Degeneration of Motoneurons in Amyotrophic Lateral Sclerosis

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Publisher
Hindawi Publishing Corporation
Copyright
Copyright © 2013 Julianne Aebischer et al.
ISSN
2090-858X
Publisher site
See Article on Publisher Site

Abstract

While studies on death receptors have long been restricted to immune cells, the last decade has provided a strong body of evidence for their implication in neuronal death and hence neurodegenerative disorders such as amyotrophic lateral sclerosis (ALS). ALS is a fatal paralytic disorder that primarily affects motoneurons in the brain and spinal cord. A neuroinflammatory process, associated with astrocyte and microglial activation as well as infiltration of immune cells, accompanies motoneuron degeneration and supports the contribution of non-cell-autonomous mechanisms in the disease. Hallmarks of Fas, TNFR, LT-βR, and p7 signaling have been observed in both animal models and ALS patients. This review summarizes to date knowledge of the role of death receptors in ALS and the link existing between the selective loss of motoneurons and neuroinflammation. It further suggests how this recent evidence could be included in an ultimate multiapproach to treat patients.

Journal

Journal of Neurodegenerative DiseasesHindawi Publishing Corporation

Published: Jul 16, 2013

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