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Neurology Genetics
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December 2020; 6 (6) Clinical/Scientific NotesOpen Access

Ketogenic diet reduces Lafora bodies in murine Lafora disease

Lori Israelian, Peixiang Wang, Shoghig Gabrielian, Xiaochu Zhao, Berge A. Minassian
First published November 19, 2020, DOI: https://doi.org/10.1212/NXG.0000000000000533
Lori Israelian
From the Institute of Medical Science (L.I.), University of Toronto, ON; Program in Genetics and Genome Biology (L.I., P.W., S.G., X.Z., B.A.M.), The Hospital for Sick Children Research Institute, Toronto, ON, Canada; and Division of Neurology (B.A.M.), Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas.
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Peixiang Wang
From the Institute of Medical Science (L.I.), University of Toronto, ON; Program in Genetics and Genome Biology (L.I., P.W., S.G., X.Z., B.A.M.), The Hospital for Sick Children Research Institute, Toronto, ON, Canada; and Division of Neurology (B.A.M.), Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas.
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Shoghig Gabrielian
From the Institute of Medical Science (L.I.), University of Toronto, ON; Program in Genetics and Genome Biology (L.I., P.W., S.G., X.Z., B.A.M.), The Hospital for Sick Children Research Institute, Toronto, ON, Canada; and Division of Neurology (B.A.M.), Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas.
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Xiaochu Zhao
From the Institute of Medical Science (L.I.), University of Toronto, ON; Program in Genetics and Genome Biology (L.I., P.W., S.G., X.Z., B.A.M.), The Hospital for Sick Children Research Institute, Toronto, ON, Canada; and Division of Neurology (B.A.M.), Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas.
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Berge A. Minassian
From the Institute of Medical Science (L.I.), University of Toronto, ON; Program in Genetics and Genome Biology (L.I., P.W., S.G., X.Z., B.A.M.), The Hospital for Sick Children Research Institute, Toronto, ON, Canada; and Division of Neurology (B.A.M.), Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas.
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Citation
Ketogenic diet reduces Lafora bodies in murine Lafora disease
Lori Israelian, Peixiang Wang, Shoghig Gabrielian, Xiaochu Zhao, Berge A. Minassian
Neurol Genet Dec 2020, 6 (6) e533; DOI: 10.1212/NXG.0000000000000533

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    Figure Blood β-hydroxybutyrate and glucose, hippocampal LB, total brain glycogen, total skeletal muscle glycogen, and brain phosphorylated GS levels in LD mice fed the ketogenic diet or normal chow

    (A) Blood β-hydroxybutyrate; time course begins 2 weeks following initiation of diet and concludes at 28 weeks; 6 mice per week. (B) Blood glucose; beginning at 1 week of diet introduction and concludes at 28 weeks; 5–8 mice per week. (C) Hippocampal LBs; 8–9 mice per group. (D) Total brain glycogen; 8–10 mice per group. (E) Skeletal muscle glycogen; 4 mice per group. Bars in A–E represent SD of the mean. Significance for all bar diagrams calculated using the unpaired Student t test; *p < 0.05; **p < 0.01. (F) Brain GS phosphorylation (inhibition) is increased in ketogenic diet–fed animals; 3 biological replicates per diet type. FW = fresh weight; GAPDH = glyceraldehyde 3-phosphate dehydrogenase; GS = glycogen synthase; LB = Lafora body; LD = Lafora disease; PASD = periodic acid–Schiff following diastase predigestion.

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links.lww.com/NXG/A337links.lww.com/NXG/A337

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