Moonwalker mouse
The Moonwalker (<em>Mwk</em>) mouse is a dominant ataxic mouse model of inherited cerebellar ataxia caused by a gain-of-function mutation in the gene encoding the TRPC3 cation channel. <em>Mwk</em> mice display overt ataxia, impaired Purkinje cell development, altered Purkinj...
| Main Authors: | , |
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| Format: | Book section |
| Language: | English |
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Springer
2023
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| _version_ | 1826342867004555264 |
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| author | Ibrahim, MF Becker, EBE |
| author2 | Gruol, DL |
| author_facet | Gruol, DL Ibrahim, MF Becker, EBE |
| author_sort | Ibrahim, MF |
| collection | OXFORD |
| description | The Moonwalker (<em>Mwk</em>) mouse is a dominant ataxic mouse model of inherited cerebellar ataxia caused by a gain-of-function mutation in the gene encoding the TRPC3 cation channel. <em>Mwk</em> mice display overt ataxia, impaired Purkinje cell development, altered Purkinje cell excitability and loss of TRPC3-expressing neurons in the cerebellum. Recently, dysregulated mGluR1-TRPC3 signalling has been implicated in multiple human spinocerebellar ataxias. Here, we discuss the behavioural, morphological, and functional changes in <em>Mwk</em> mice with an emphasis on their relevance to the human spinocerebellar ataxias. |
| first_indexed | 2025-03-11T23:38:05Z |
| format | Book section |
| id | oxford-uuid:6e3832d8-3355-42fb-a63a-8304f37ccbf5 |
| institution | University of Oxford |
| language | English |
| last_indexed | 2025-03-11T23:38:05Z |
| publishDate | 2023 |
| publisher | Springer |
| record_format | dspace |
| spelling | oxford-uuid:6e3832d8-3355-42fb-a63a-8304f37ccbf52025-03-11T12:12:15ZMoonwalker mouseBook sectionhttp://purl.org/coar/resource_type/c_1843uuid:6e3832d8-3355-42fb-a63a-8304f37ccbf5EnglishSymplectic ElementsSpringer2023Ibrahim, MFBecker, EBEGruol, DLKoibuchi, NManto, MMolinari, MSchmahmann, JDShen, YThe Moonwalker (<em>Mwk</em>) mouse is a dominant ataxic mouse model of inherited cerebellar ataxia caused by a gain-of-function mutation in the gene encoding the TRPC3 cation channel. <em>Mwk</em> mice display overt ataxia, impaired Purkinje cell development, altered Purkinje cell excitability and loss of TRPC3-expressing neurons in the cerebellum. Recently, dysregulated mGluR1-TRPC3 signalling has been implicated in multiple human spinocerebellar ataxias. Here, we discuss the behavioural, morphological, and functional changes in <em>Mwk</em> mice with an emphasis on their relevance to the human spinocerebellar ataxias. |
| spellingShingle | Ibrahim, MF Becker, EBE Moonwalker mouse |
| title | Moonwalker mouse |
| title_full | Moonwalker mouse |
| title_fullStr | Moonwalker mouse |
| title_full_unstemmed | Moonwalker mouse |
| title_short | Moonwalker mouse |
| title_sort | moonwalker mouse |
| work_keys_str_mv | AT ibrahimmf moonwalkermouse AT beckerebe moonwalkermouse |