An inducible glycogen synthase-1 knockout halts but does not reverse Lafora disease progression in mice

Malstructured glycogen accumulates over time in Lafora disease (LD) and precipitates into Lafora bodies (LBs), leading to neurodegeneration and intractable fatal epilepsy. Constitutive reduction of glycogen synthase-1 (GYS1) activity prevents murine LD, but the effect of GYS1 reduction later in dise...

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Detalles Bibliográficos
Autores principales: Nitschke, Silvia, Chown, Erin E., Zhao, Xiaochu, Gabrielian, Shoghig, Petković, Sara, Guisso, Dikran R., Perri, Ami M., Wang, Peixiang, Ahonen, Saija, Nitschke, Felix, Minassian, Berge A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Society for Biochemistry and Molecular Biology 2020
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7857511/
https://www.ncbi.nlm.nih.gov/pubmed/33277363
http://dx.doi.org/10.1074/jbc.RA120.015773

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7857511/
https://www.ncbi.nlm.nih.gov/pubmed/33277363
http://dx.doi.org/10.1074/jbc.RA120.015773

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