Literature DB >> 21930129

Are there errors in glycogen biosynthesis and is laforin a repair enzyme?

Peter J Roach1.   

Abstract

Glycogen, a branched polymer of glucose, is well known as a cellular reserve of metabolic energy and/or biosynthetic precursors. Besides glucose, however, glycogen contains small amounts of covalent phosphate, present as C2 and C3 phosphomonoesters. Current evidence suggests that the phosphate is introduced by the biosynthetic enzyme glycogen synthase as a rare alternative to its normal catalytic addition of glucose units. The phosphate can be removed by the laforin phosphatase, whose mutation causes a fatal myoclonus epilepsy called Lafora disease. The hypothesis is that glycogen phosphorylation can be considered a catalytic error and laforin a repair enzyme.
Copyright © 2011 Federation of European Biochemical Societies. Published by Elsevier B.V. All rights reserved.

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Year:  2011        PMID: 21930129      PMCID: PMC4939770          DOI: 10.1016/j.febslet.2011.09.009

Source DB:  PubMed          Journal:  FEBS Lett        ISSN: 0014-5793            Impact factor:   4.124


  26 in total

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Journal:  FEBS Lett       Date:  1970-12-28       Impact factor: 4.124

Review 2.  Lafora disease, seizures and sugars.

Authors:  D M Andrade; J Turnbull; B A Minassian
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3.  Phosphate incorporation during glycogen synthesis and Lafora disease.

Authors:  Vincent S Tagliabracci; Christian Heiss; Chandra Karthik; Christopher J Contreras; John Glushka; Mayumi Ishihara; Parastoo Azadi; Thomas D Hurley; Anna A DePaoli-Roach; Peter J Roach
Journal:  Cell Metab       Date:  2011-03-02       Impact factor: 27.287

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Journal:  FEBS Lett       Date:  1980-01-01       Impact factor: 4.124

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Journal:  Biofactors       Date:  1994-05       Impact factor: 6.113

6.  Requirements for catalysis in mammalian glycogenin.

Authors:  Thomas D Hurley; Stephanie Stout; Emily Miner; Jing Zhou; Peter J Roach
Journal:  J Biol Chem       Date:  2005-04-22       Impact factor: 5.157

Review 7.  Fidelity at the molecular level: lessons from protein synthesis.

Authors:  Hani S Zaher; Rachel Green
Journal:  Cell       Date:  2009-02-20       Impact factor: 41.582

8.  Laforin is a glycogen phosphatase, deficiency of which leads to elevated phosphorylation of glycogen in vivo.

Authors:  Vincent S Tagliabracci; Julie Turnbull; Wei Wang; Jean-Marie Girard; Xiaochu Zhao; Alexander V Skurat; Antonio V Delgado-Escueta; Berge A Minassian; Anna A Depaoli-Roach; Peter J Roach
Journal:  Proc Natl Acad Sci U S A       Date:  2007-11-26       Impact factor: 11.205

Review 9.  The autosomal recessively inherited progressive myoclonus epilepsies and their genes.

Authors:  Nivetha Ramachandran; Jean-Marie Girard; Julie Turnbull; Berge A Minassian
Journal:  Epilepsia       Date:  2009-05       Impact factor: 5.864

Review 10.  Medium- and short-chain dehydrogenase/reductase gene and protein families : the MDR superfamily.

Authors:  B Persson; J Hedlund; H Jörnvall
Journal:  Cell Mol Life Sci       Date:  2008-12       Impact factor: 9.261

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  8 in total

Review 1.  Glycogen phosphorylation and Lafora disease.

Authors:  Peter J Roach
Journal:  Mol Aspects Med       Date:  2015-08-13

Review 2.  Protein tyrosine phosphatases--from housekeeping enzymes to master regulators of signal transduction.

Authors:  Nicholas K Tonks
Journal:  FEBS J       Date:  2013-01-17       Impact factor: 5.542

Review 3.  Lafora disease offers a unique window into neuronal glycogen metabolism.

Authors:  Matthew S Gentry; Joan J Guinovart; Berge A Minassian; Peter J Roach; Jose M Serratosa
Journal:  J Biol Chem       Date:  2018-02-26       Impact factor: 5.157

Review 4.  Lafora disease: from genotype to phenotype.

Authors:  Rashmi Parihar; Anupama Rai; Subramaniam Ganesh
Journal:  J Genet       Date:  2018-07       Impact factor: 1.166

Review 5.  Does abnormal glycogen structure contribute to increased susceptibility to seizures in epilepsy?

Authors:  Mauro DiNuzzo; Silvia Mangia; Bruno Maraviglia; Federico Giove
Journal:  Metab Brain Dis       Date:  2014-03-19       Impact factor: 3.584

6.  Dimerization of the glucan phosphatase laforin requires the participation of cysteine 329.

Authors:  Pablo Sánchez-Martín; Madushi Raththagala; Travis M Bridges; Satrio Husodo; Matthew S Gentry; Pascual Sanz; Carlos Romá-Mateo
Journal:  PLoS One       Date:  2013-07-26       Impact factor: 3.240

7.  The carbohydrate-binding domain of overexpressed STBD1 is important for its stability and protein-protein interactions.

Authors:  Yuanqi Zhu; Mei Zhang; Amber Renee Kelly; Alan Cheng
Journal:  Biosci Rep       Date:  2014-07-01       Impact factor: 3.840

8.  Expression, purification and characterization of soluble red rooster laforin as a fusion protein in Escherichia coli.

Authors:  M Kathryn Brewer; Satrio Husodo; Vikas V Dukhande; Mary Beth Johnson; Matthew S Gentry
Journal:  BMC Biochem       Date:  2014-04-02       Impact factor: 4.059

  8 in total

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