The pathogenesis of amyotrophic lateral sclerosis and frontal lobe dementia is unraveling : pathology of the nucleus and glutamate sensitivity
The mechanisms of neurodegenerative diseases have begun to become unraveled, thanks to the progress in stem cell research. The repeat expansion in the C90RF72 gene was identified in 2011 as the most common genetic cause of both ALS and frontal lobe dementia. Only over a couple of years the disease mechanisms of this mutation have been revealed and treatment trials have already been conducted in nerve cell cultures differentiated from patients' stem cells. We discuss the role of the repeat expansion in the C90RF72 gene in the epidemiology of the diseases and the resulting disturbances in nerve cell function.
| Medienart: |
Artikel |
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| Erscheinungsjahr: |
2016 |
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| Erschienen: |
2016 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:132 |
|---|---|
| Enthalten in: |
Duodecim; laaketieteellinen aikakauskirja - 132(2016), 5 vom: 02., Seite 423-31 |
| Sprache: |
Finnisch |
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| Weiterer Titel: |
Amyotrofisen lateraaliskleroosin ja otsalohkodementian patogeneesi avautuu: Tumapatologia ja glutamaattiherkkyys |
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| Beteiligte Personen: |
Tienari, Pentti [VerfasserIn] |
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| Themen: |
3KX376GY7L |
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| Anmerkungen: |
Date Completed 12.05.2016 Date Revised 16.11.2017 published: Print Citation Status MEDLINE |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM259508292 |
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| 245 | 1 | 4 | |a The pathogenesis of amyotrophic lateral sclerosis and frontal lobe dementia is unraveling |b pathology of the nucleus and glutamate sensitivity |
| 246 | 3 | 3 | |a Amyotrofisen lateraaliskleroosin ja otsalohkodementian patogeneesi avautuu: Tumapatologia ja glutamaattiherkkyys |
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| 520 | |a The mechanisms of neurodegenerative diseases have begun to become unraveled, thanks to the progress in stem cell research. The repeat expansion in the C90RF72 gene was identified in 2011 as the most common genetic cause of both ALS and frontal lobe dementia. Only over a couple of years the disease mechanisms of this mutation have been revealed and treatment trials have already been conducted in nerve cell cultures differentiated from patients' stem cells. We discuss the role of the repeat expansion in the C90RF72 gene in the epidemiology of the diseases and the resulting disturbances in nerve cell function | ||
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| 700 | 1 | |a Laaksovirta, Hannu |e verfasserin |4 aut | |
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