Glial Alterations in the Glutamatergic and GABAergic Signalling Pathways in a Mouse Model of Lafora Disease, a Severe Form of Progressive Myoclonus Epilepsy

The observed upregulation of glutamatergic receptor subunits likely amplifies excitatory glutamatergic signalling, whereas the increased expression of GAT1 may reduce the inhibitory GABAergic tone. These changes contribute to the characteristic hyperexcitability of LD.

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Publication Details

Journal
Neuropathology and Applied Neurobiology
Published
2025-03-04
DOI
https://doi.org/10.1111/nan.70009
Citations
1
Primary Topic
Glycogen Storage Diseases and Myoclonus
Type
article
Field-Weighted Citation Impact
1.67

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article

Glial Alterations in the Glutamatergic and GABAergic Signalling Pathways in a Mouse Model of Lafora Disease, a Severe Form of Progressive Myoclonus Epilepsy

Ángela Campos‐Rodríguez, Pascual Sanz, Rosa Viana, Teresa Rubio
1 citations
Neuropathology and Applied Neurobiology
Glycogen Storage Diseases and Myoclonus
1.67
article

Glial Alterations in the Glutamatergic and GABAergic Signalling Pathways in a Mouse Model of Lafora Disease, a Severe Form of Progressive Myoclonus Epilepsy

Ángela Campos‐Rodríguez, Pascual Sanz, Rosa Viana, Teresa Rubio
article en
1 citations

Abstract

The observed upregulation of glutamatergic receptor subunits likely amplifies excitatory glutamatergic signalling, whereas the increased expression of GAT1 may reduce the inhibitory GABAergic tone. These changes contribute to the characteristic hyperexcitability of LD.

Neuropathology and Applied NeurobiologyVol. 51(2)
Instituto de Salud Carlos III (ES), Centre for Biomedical Network Research on Rare Diseases (ES), Instituto de Biomedicina de Valencia (ES), Instituto de Investigación de Enfermedades Raras (ES), Universidad Europea de Madrid (ES)
Fundació la Marató de TV3, Generalitat Valenciana, Ministerio de Ciencia y Tecnología, National Institutes of Health
Openalex Percentile: Top 20%
Glycogen Storage Diseases and Myoclonus
1.67
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