EGFR inhibitor erlotinib delays disease progression but does not extend survival in the SOD1 mouse model of ALS.
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that causes progressive paralysis due to motor neuron death. Several lines of published evidence suggested that inhibition of epidermal growth factor receptor (EGFR) signaling might protect neurons from degeneration. To test th...
| Main Authors: | , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Public Library of Science (PLoS)
2013-01-01
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| Series: | PLoS ONE |
| Online Access: | http://europepmc.org/articles/PMC3637182?pdf=render |
| _version_ | 1818273871048998912 |
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| author | Claire E Le Pichon Sara L Dominguez Hilda Solanoy Hai Ngu Nicholas Lewin-Koh Mark Chen Jeffrey Eastham-Anderson Ryan Watts Kimberly Scearce-Levie |
| author_facet | Claire E Le Pichon Sara L Dominguez Hilda Solanoy Hai Ngu Nicholas Lewin-Koh Mark Chen Jeffrey Eastham-Anderson Ryan Watts Kimberly Scearce-Levie |
| author_sort | Claire E Le Pichon |
| collection | DOAJ |
| description | Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that causes progressive paralysis due to motor neuron death. Several lines of published evidence suggested that inhibition of epidermal growth factor receptor (EGFR) signaling might protect neurons from degeneration. To test this hypothesis in vivo, we treated the SOD1 transgenic mouse model of ALS with erlotinib, an EGFR inhibitor clinically approved for oncology indications. Although erlotinib failed to extend ALS mouse survival it did provide a modest but significant delay in the onset of multiple behavioral measures of disease progression. However, given the lack of protection of motor neuron synapses and the lack of survival extension, the small benefits observed after erlotinib treatment appear purely symptomatic, with no modification of disease course. |
| first_indexed | 2024-12-12T22:04:50Z |
| format | Article |
| id | doaj.art-54e00785221c4de6b298184db08a7ae1 |
| institution | Directory Open Access Journal |
| issn | 1932-6203 |
| language | English |
| last_indexed | 2024-12-12T22:04:50Z |
| publishDate | 2013-01-01 |
| publisher | Public Library of Science (PLoS) |
| record_format | Article |
| series | PLoS ONE |
| spelling | doaj.art-54e00785221c4de6b298184db08a7ae12022-12-22T00:10:24ZengPublic Library of Science (PLoS)PLoS ONE1932-62032013-01-0184e6234210.1371/journal.pone.0062342EGFR inhibitor erlotinib delays disease progression but does not extend survival in the SOD1 mouse model of ALS.Claire E Le PichonSara L DominguezHilda SolanoyHai NguNicholas Lewin-KohMark ChenJeffrey Eastham-AndersonRyan WattsKimberly Scearce-LevieAmyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that causes progressive paralysis due to motor neuron death. Several lines of published evidence suggested that inhibition of epidermal growth factor receptor (EGFR) signaling might protect neurons from degeneration. To test this hypothesis in vivo, we treated the SOD1 transgenic mouse model of ALS with erlotinib, an EGFR inhibitor clinically approved for oncology indications. Although erlotinib failed to extend ALS mouse survival it did provide a modest but significant delay in the onset of multiple behavioral measures of disease progression. However, given the lack of protection of motor neuron synapses and the lack of survival extension, the small benefits observed after erlotinib treatment appear purely symptomatic, with no modification of disease course.http://europepmc.org/articles/PMC3637182?pdf=render |
| spellingShingle | Claire E Le Pichon Sara L Dominguez Hilda Solanoy Hai Ngu Nicholas Lewin-Koh Mark Chen Jeffrey Eastham-Anderson Ryan Watts Kimberly Scearce-Levie EGFR inhibitor erlotinib delays disease progression but does not extend survival in the SOD1 mouse model of ALS. PLoS ONE |
| title | EGFR inhibitor erlotinib delays disease progression but does not extend survival in the SOD1 mouse model of ALS. |
| title_full | EGFR inhibitor erlotinib delays disease progression but does not extend survival in the SOD1 mouse model of ALS. |
| title_fullStr | EGFR inhibitor erlotinib delays disease progression but does not extend survival in the SOD1 mouse model of ALS. |
| title_full_unstemmed | EGFR inhibitor erlotinib delays disease progression but does not extend survival in the SOD1 mouse model of ALS. |
| title_short | EGFR inhibitor erlotinib delays disease progression but does not extend survival in the SOD1 mouse model of ALS. |
| title_sort | egfr inhibitor erlotinib delays disease progression but does not extend survival in the sod1 mouse model of als |
| url | http://europepmc.org/articles/PMC3637182?pdf=render |
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