| Autor: |
Paul H. Gordon, Caterina Bendotti, Jacqueline Montes, Sari Goren, Zipora Speiser, Cristina Cheroni, Eran Blaugrund, Massimo Tortarolo, Hussein Hallak |
| Rok vydání: |
2008 |
| Předmět: |
|
| DOI: |
10.1016/b978-0-12-373861-5.00019-9 |
| Popis: |
Publisher Summary Amyotrophic Lateral Sclerosis (ALS) is an age-related neurodegenerative disease affecting Motor Neurons (MNs). There is no known cure and survival extends approximately 3 years on average from symptom onset. Nearly two decades ago, the discovery that mutations in the Cu/Zn Superoxide Dismutase (SOD1) gene cause ALS in some families led to the advent of a transgenic rodent model. Since then, several other chromosomal loci have been identified, but the majority of ALS cases are thought to be sporadic. Recent advances in the understanding of the pathogenesis have prompted numerous clinical trials of investigational agents and advanced scientific methods are being used to examine the neuroprotective properties of large quantities of compounds. Yet, despite great strides in both the basic and clinical sciences, progress in ALS research so far has not translated into practical clinical applications. ALS researchers are working toward innovative models testing new mechanisms of cell degeneration, genome wide screens, and novel therapies for the future. ALS offers the chance of proof-of-concept studies faster than other disorders. The rapidly progressive disease course makes ALS a good testing ground for new therapies. |
| Databáze: |
OpenAIRE |
| Externí odkaz: |
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