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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Vydáno v:J Biol Chem
Hlavní autoři: 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.
Médium: Artigo
Jazyk:Inglês
Vydáno: American Society for Biochemistry and Molecular Biology 2020
Témata:
Research Article
On-line přístup:https://ncbi.nlm.nih.gov/pmc/articles/PMC7857511/
https://ncbi.nlm.nih.gov/pubmed/33277363
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.RA120.015773
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