Alleviation of a polyglucosan storage disorder by enhancement of autophagic glycogen catabolism

Alleviation of a polyglucosan storage disorder by enhancement of autophagic glycogen catabolism

Abstract This work employs adult polyglucosan body disease (APBD) models to explore the efficacy and mechanism of action of the polyglucosan‐reducing compound 144DG11. APBD is a glycogen storage disorder (GSD) caused by glycogen branching enzyme (GBE) deficiency causing accumulation of poorly branch...

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Bibliographic Details
Main Authors: Or Kakhlon, Hilla Vaknin, Kumudesh Mishra, Jeevitha D’Souza, Monzer Marisat, Uri Sprecher, Shane Wald‐Altman, Anna Dukhovny, Yuval Raviv, Benny Da’adoosh, Hamutal Engel, Sandrine Benhamron, Keren Nitzan, Sahar Sweetat, Anna Permyakova, Anat Mordechai, Hasan Orhan Akman, Hanna Rosenmann, Alexander Lossos, Joseph Tam, Berge A. Minassian, Miguel Weil
Format: Article
Language:English
Published: Springer Nature 2021-09-01
Series:EMBO Molecular Medicine
Subjects:
adult polyglucosan body disease
autophagy
glycogen
lysosomes
polyglucosan
Online Access:https://doi.org/10.15252/emmm.202114554

Internet

https://doi.org/10.15252/emmm.202114554

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