ArticleMolecular cell2015
Structural mechanism of laforin function in glycogen dephosphorylation and lafora disease.
Article in Molecular cell, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 37 papers.
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Who cites it
37 citing papers in PubMed, 58 citations in OpenAlex.
- Discovery and Evaluation of Active Site-Directed, Potent, and Selective Sulfophenyl Acetic Amide-Based Inhibitors for the Laforin Phosphatase.Journal of medicinal chemistry · 2025Article
- Identification of Novel Inhibitors of Starch Excess 4 (SEX4).Life (Basel, Switzerland) · 2024Article
- Development of Novel Phosphonodifluoromethyl-Containing Phosphotyrosine Mimetics and a First-In-Class, Potent, Selective, and Bioavailable Inhibitor of Human CDC14 Phosphatases.Journal of medicinal chemistry · 2024Article
- The multifaceted roles of the brain glycogen.Journal of neurochemistry · 2024Review
- Inhibition of a conserved bacterial dual-specificity phosphatase confers plant tolerance toiScience · 2024Article
- Lafora progressive myoclonus epilepsy: Disease mechanism and therapeutic attempts.Journal of biosciences · 2024Review
- The LIKE SEX FOUR 1-malate dehydrogenase complex functions as a scaffold to recruit β-amylase to promote starch degradation.The Plant cell · 2023Article
- Gys1 Antisense Therapy Prevents Disease-Driving Aggregates and Epileptiform Discharges in a Lafora Disease Mouse Model.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2023Article
- Transcriptomes of Clusterin- and S100B-transfected neuronal cells elucidate protective mechanisms against hypoxia and oxidative stress in the hooded seal (Cystophora cristata) brain.BMC neuroscience · 2022Article
- Novel mutation of EPM2A causes progressive myoclonic epilepsy: a case report.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2022Article
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- Generation and characterization of a laforin nanobody inhibitor.Clinical biochemistry · 2021Article
- A novel gene therapy for neurodegenerative Lafora disease viaNanomedicine (London, England) · 2021Article
- Genetic cause of epilepsy in a Greek cohort of children and young adults with heterogeneous epilepsy syndromes.Epilepsy & behavior reports · 2021Article
- Polyglucosan body structure in Lafora disease.Carbohydrate polymers · 2020Article
- Central Nervous System Delivery and Biodistribution Analysis of an Antibody-Enzyme Fusion for the Treatment of Lafora Disease.Molecular pharmaceutics · 2019Article
- Molecular Architecture of the Inositol Phosphatase Siw14.Biochemistry · 2019Article
- Brain Glycogen Structure and Its Associated Proteins: Past, Present and Future.Advances in neurobiology · 2019Review
- Oligomerization and carbohydrate binding of glucan phosphatases.Analytical biochemistry · 2018Article
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Authors and funding
21 authors at 5 institutions in 2 countries.
Funding
Abstract
Glycogen is the major mammalian glucose storage cache and is critical for energy homeostasis. Glycogen synthesis in neurons must be tightly controlled due to neuronal sensitivity to perturbations in glycogen metabolism. Lafora disease (LD) is a fatal, congenital, neurodegenerative epilepsy. Mutations in the gene encoding the glycogen phosphatase laforin result in hyperphosphorylated glycogen that forms water-insoluble inclusions called Lafora bodies (LBs). LBs induce neuronal apoptosis and are the causative agent of LD. The mechanism of glycogen dephosphorylation by laforin and dysfunction in LD is unknown. We report the crystal structure of laforin bound to phosphoglucan product, revealing its unique integrated tertiary and quaternary structure. Structure-guided mutagenesis combined with biophysical and biochemical analyses reveal the basis for normal function of laforin in glycogen metabolism. Analyses of LD patient mutations define the mechanism by which subsets of mutations disrupt laforin function. These data provide fundamental insights connecting glycogen metabolism to neurodegenerative disease.
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