Publications

All Publications (newest to oldest)

• Therapeutic Window for the Treatment of Lafora Disease

Varea O, Guinovart JJ, Duran J

Jasper's Basic Mechanisms of the Epilepsies. 2024. doi: https://doi.org/10.1093/med/9780197549469.003.0053 

• Brain energy metabolism: A roadmap for future research

Rae CD, Baur JA, Borges K, Dienel G, Díaz-García CM, Douglass SR, Drew K, Duarte JMN, Duran J et al.

Journal of Neurochemistry. 2024. doi: https://doi.org/10.1111/jnc.16032  

PMID: 38183680

• Glycogen accumulation modulates life span in a mouse model of amyotrophic lateral sclerosis

M. Kathryn Brewer, Pascual Torres, Victòria Ayala, Manuel Portero-Otin, Reinald Pamplona, Pol Andrés-Benito, Isidro Ferrer, Joan J. Guinovart, Duran J

Journal of Neurochemistry. 2023. doi: https://doi.org/10.1111/jnc.15906

PMID: 37401737  

• Mutant IDH regulates glycogen metabolism from early cartilage development to malignant chondrosarcoma formation.

Pathmanapan S, Poon R, De Renshaw TB, Nadesan P, Nakagawa M, Seesankar GA, Ho Loe AK, Zhang HH, Guinovart JJ, Duran J, Newgard CB, Wunder JS, Alman BA.

Cell Rep. 2023 Jun 1;42(6):112578. https://doi.org/10.1016/j.celrep.2023.112578

PMID: 37267108  

• Role of Astrocytes in the Pathophysiology of Lafora Disease and Other Glycogen Storage Disorders

Duran J

Cells. 2023 Feb 24;12(5):722. doi: https://doi.org/10.3390/cells12050722

PMID: 36899857 

• Active Glycogen Synthase in the Liver Prevents High-Fat Diet-Induced Glucose Intolerance, Decreases Food Intake, and Lowers Body Weight.

López-Soldado I, Guinovart JJ, Duran J.

Int J Mol Sci. 2023 Jan 29;24(3):2574. doi: https://doi.org/10.3390/ijms24032574   

PMID: 36768897

• Hepatic overexpression of protein targeting to glycogen attenuates obesity and improves hyperglycemia in db/db mice.

López-Soldado I, Guinovart JJ, Duran J.

Front Endocrinol (Lausanne). 2022 Sep 9;13:969924. doi: https://doi.org/10.3389/fendo.2022.969924 

PMID: 36157460

• Malin restoration as proof of concept for gene therapy for Lafora disease.

Varea O, Guinovart JJ, Duran J.

Brain Commun. 2022 Jun 23;4(4):fcac168. doi: https://doi.org/10.1093/braincomms/fcac168 

PMID: 35813879

• Lack of p62 Impairs Glycogen Aggregation and Exacerbates Pathology in a Mouse Model of Myoclonic Epilepsy of Lafora.

Pellegrini P, Hervera A, Varea O, Brewer MK, López-Soldado I, Guitart A, Aguilera M, Prats N, Del Río JA, Guinovart JJ, Duran J.

Mol Neurobiol. 2022 Feb;59(2):1214-1229. doi: https://doi.org/10.1007/s12035-021-02682-6 

PMID: 34962634

• p38γ and p38δ regulate postnatal cardiac metabolism through glycogen synthase 1.

Santamans AM, Montalvo-Romeral V, Mora A et al (Duran J 12/20)

PLoS Biol. 2021 Nov 10;19(11):e3001447. doi: https://doi.org/10.1371/journal.pbio.3001447 

PMID: 34758018

• Increased liver glycogen levels enhance exercise capacity in mice.

López-Soldado I, Guinovart JJ, Duran J.

J Biol Chem. 2021 Aug;297(2):100976. doi: https://doi.org/10.1016/j.jbc.2021.100976 

PMID: 34284060

• Neutrophils fuel effective immune responses through gluconeogenesis and glycogenesis.

Sadiku P, Willson JA, Ryan EM, Sammut D et al (Duran J 26/37)

Cell Metab. 2021 May 4;33(5):1062-1064. doi: https://doi.org/10.1016/j.cmet.2021.03.018 

PMID: 33951466

• Alteration of mitochondrial function in the livers of mice with glycogen branching enzyme deficiency.

Malinska D, Testoni G, Bejtka M, Duran J, Guinovart JJ, Duszynski J.

Biochimie. 2021 Jul;186:28-32. doi: https://doi.org/10.1016/j.biochi.2021.04.001 

PMID: 33857563

• Astrocytic glycogen accumulation drives the pathophysiology of neurodegeneration in Lafora disease.

Duran J*, Hervera A, Markussen KH, Varea O, López-Soldado I, Sun RC, del Río JA, Gentry MS, Guinovart JJ

Brain. 2021 Sep 4;144(8):2349-2360. doi: https://doi.org/10.1093/brain/awab110 

PMID: 33822008

*corresponding author

• Alteration of mitochondrial function in the livers of mice with glycogen branching enzyme deficiency.

Malinska D, Testoni G, Bejtka M, Duran J, Guinovart JJ, Duszynski J.

Biochimie. 2021 Jul;186:28-32. doi: https://doi.org/10.1016/j.biochi.2021.04.001 

PMID: 33857563

• Increasing hepatic glycogen moderates the diabetic phenotype in insulin-deficient Akita mice

López-Soldado I, Guinovart JJ, Duran J

JBC. 2021 Mar 2;296:100498. doi: https://doi.org/10.1016/j.jbc.2021.100498 

PMID: 33667544

• Pulmonary glycogen deficiency as a new potential cause of Respiratory Distress Syndrome.

Testoni G, Olmeda B, Duran J, López-Rodríguez E, Aguilera M, Hernández-Álvarez MI, Prats N, Pérez-Gil J, Guinovart JJ.

Hum Mol Genet. 2021 Jan 6;29(21):3554-3565. doi: https://doi.org/10.1093/hmg/ddaa249 

PMID: 33219378

• Neutrophils fuel effective immune responses through gluconeogenesis and glycogenesis.

Sadiku P, Willson JA, Ryan EM, et al (Duran J 26/37)

Cell Metab. 2020 Dec 1:S1550-4131(20)30651-3. doi: https://doi.org/10.1016/j.cmet.2020.11.016 

PMID: 33306983

• Suppression of glycogen synthesis as a treatment for Lafora disease: Establishing the window of opportunity.

Varea O, Duran J*, Aguilera M, Prats N, Guinovart JJ.

Neurobiol Dis. 2020 Nov 7;147:105173. doi: https://doi.org/10.1016/j.nbd.2020.105173 

PMID: 33171226

 *corresponding author

• Hallmarks of oxidative stress in the livers of aged mice with mild glycogen branching enzyme deficiency.

Malinska D, Testoni G, Duran J, Brudnicka A, Guinovart JJ, Duszynski J.

Arch Biochem Biophys. 2020 Nov 30;695:108626. doi: https://doi.org/10.1016/j.abb.2020.108626 

PMID: 33049291

• Lack of Astrocytic Glycogen Alters Synaptic Plasticity but Not Seizure Susceptibility.

Duran J*, Brewer MK, Hervera A, Gruart A, Del Rio JA, Delgado-García JM, Guinovart JJ.Mol Neurobiol. 2020 Nov;57(11):4657-4666. doi: https://doi.org/10.1007/s12035-020-02055-5 

PMID: 32770452

 *corresponding author

• Maintenance of liver glycogen during long-term fasting preserves energy state in mice.

López-Soldado I, Bertini A, Adrover A, Duran J, Guinovart JJ.

FEBS Letters. 2020 Jun;594(11):1698-1710. doi: https://doi.org/10.1002/1873-3468.13770 

PMID: 32159852

• Arousal-induced cortical activity triggers lactate release from astrocytes.

Zuend M, Saab AS, Wyss MT, Ferrari KD, Hösli L, Looser ZJ, Stobart JL, Duran J, Guinovart JJ, Barros LF and Weber B.

Nat Metab. 2020; 2:179–191. doi: https://doi.org/10.1038/s42255-020-0170-4 

PMID: 32694692

• Glycogen in Astrocytes and Neurons: Physiological and Pathological Aspects.

Duran J, Gruart A, López-Ramos JC, Delgado-García JM, Guinovart JJ.

Adv Neurobiol. 2019;23:311-329. doi: https://doi.org/10.1007/978-3-030-27480-1_10 

PMID: 31667813

• Lack of Neuronal Glycogen Impairs Memory Formation and Learning-Dependent Synaptic Plasticity in Mice.

Duran J, Gruart A, Varea O, López-Soldado I, Delgado-García JM, Guinovart JJ.

Front Cell Neurosci. 2019 Aug 13;13:374. doi: https://doi.org/10.3389/fncel.2019.00374 

PMID: 31456667

• Exploring the elusive composition of corpora amylacea of human brain. Augé E, Duran J, Guinovart JJ, Pelegrí C, Vilaplana J.

Sci Rep. 2018 Sep 10;8(1):13525. doi: https://doi.org/10.1038/s41598-018-31766-y 

PMID: 30202002

• Astrocytes and neurons produce distinct types of polyglucosan bodies in Lafora disease.

Augé E, Pelegrí C, Manich G, Cabezón I, Guinovart JJ, Duran J*, Vilaplana J*.

Glia. 2018 Oct;66(10):2094-2107. doi: https://doi.org/10.1002/glia.23463 

PMID: 30152044

*corresponding authors

• Lack of glycogenin causes glycogen accumulation and muscle function impairment

Testoni G, Duran J, García-Rocha M, Vilaplana F, Serrano AL, Sebastián D, Lopez-Soldado I, Sullivan MA, Slebe F, Vilaseca M, Muñoz-Cánoves P, Guinovart JJ.

Cell Metabolism. 2017 Jul 5;26(1):256-266.e4. doi: https://doi.org/10.1016/j.cmet.2017.06.008 

PMID: 28683291

• Effects of hepatic glycogen on food intake and glucose homeostasis are mediated by the vagus nerve in mice.

López-Soldado I, Fuentes-Romero R, Duran J, Guinovart JJ.

Diabetologia. 2017 Jun;60(6):1076-1083. doi: https://doi.org/10.1007/s00125-017-4240-4 

PMID: 28299379

• Differential glucose metabolism in mice and humans affected by McArdle disease.

Krag TO, Pinós T, Nielsen TL, Duran J, García-Rocha M, Andreu AL, Vissing J.

Am J Physiol Regul Integr Comp Physiol. 2016 Aug 1;311(2):R307-14. doi: http://doi.org/10.1152/ajpregu.00489.2015 

PMID: 27280431 

• Genetic models rule out a major role of beta cell glycogen in the control of glucose homeostasis.

Mir-Coll J, Duran J, Slebe F, García-Rocha M,Gomis R,Gasa R, Guinovart JJ.

Diabetologia. 2016 May;59(5):1012-20. doi: https://doi.org/10.1007/s00125-016-3871-1 

PMID: 26825527

• Brain glycogen in health and disease.

Duran J, Guinovart JJ.

Mol Aspects Med. 2015 Dec;46:70-7. doi: https://doi.org/10.1016/j.mam.2015.08.007 

PMID: 26344371

• Role of brain glycogen in the response to hypoxia and in the susceptibility to epilepsy

López-Ramos JC*, Duran J*, Gruart A, Guinovart JJ, Delgado-García JM.

Front Cell Neurosci. 2015 Oct 27;9:431. doi: https://doi.org/10.3389/fncel.2015.00431 

PMID: 26578889

* co-authors

• Liver glycogen reduces food intake and attenuates obesity in a high-fat diet-fed mouse model.

López-Soldado I, Zafra D, Duran J, Adrover A, Calbó J, Guinovart JJ.

Diabetes. 2015 Mar;64(3):796-807. doi: https://doi.org/10.2337/db14-0728 

PMID: 25277398

• Neuronal glycogen synthesis contributes to physiological aging.

Sinadinos C, Valles-Ortega J, Boulan L, Solsona E, Tevy MF, Marquez M, Duran J, Lopez-Iglesias C, Calbó J, Blasco E, Pumarola M, Milán M, Guinovart JJ.

Aging Cell. 2014 Oct;13(5):935-45. doi: https://doi.org/10.1111/acel.12254  

PMID: 25059425

• Glycogen accumulation underlies neurodegeneration and autophagy impairment in Lafora disease.

Duran J, Gruart A, García-Rocha M, Delgado-García JM, Guinovart J.

Hum Mol Genet. 2014 Jun 15;23(12):3147-56. doi: https://doi.org/10.1093/hmg/ddu024 

PMID: 24452334

• Neurons have an active glycogen metabolism that contributes to tolerance to hypoxia

Saez I, Duran J, Sinadinos C, Yanes O, Martínez-Pons C, Tevy F, Milan M, Guinovart J.

J Cereb Blood Flow Metab. 2014 Jun;34(6):945-55 doi: https://doi.org/10.1038/jcbfm.2014.33 

PMID: 24569689

• Autophagy-regulating TP53INP2 mediates muscle wasting and is repressed in diabetes.

Sala D, Ivanova S, Plana N, Ribas V, Duran J, Bach D, Turkseven S, Laville M, Vidal H, Karczewska-Kupczewska M, Kowalska I, Straczkowski M, Testar X, Palacín M, Sandri M, Serrano AL, Zorzano A.

J Clin Invest. 2014 May;124(5):1914-27. doi: https://doi.org/10.1172/JCI72327  

PMID: 24713655

• Muscle glycogen synthase isoform is responsible for testicular glycogen synthesis: glycogen overproduction induces apoptosis in male germ cells.

Villarroel-Espíndola F, Maldonado R, Mancilla H, vander Stelt K, Acuña AI, Covarrubias A, López C, Angulo C, Castro MA, Slebe JC, Duran J, García-Rocha M, Guinovart JJ, Concha II. 

J Cell Biochem. 2013 Jul;114(7):1653-64. doi: https://doi.org/10.1002/jcb.24507.

PMID: 23386391

• Impairment in long-term memory formation and learning-dependent synaptic plasticity in mice lacking Glycogen Synthase in the brain.

Duran J, Saez I, Gruart A, Guinovart JJ, Delgado-García JM.

J Cereb Blood Flow Metab. 2013 Apr;33(4):550-6. doi: https://doi.org/10.1038/jcbfm.2012.200 

PMID: 23281428

• A Guideline to Univariate Statistical Analysis for LC/MS-Based Untargeted Metabolomics-Derived Data.

Vinaixa M, Samino S, Saez I, Duran J, Guinovart JJ, Yanes O.

Metabolites. 2012 Oct 18;2(4):775-95. doi: https://doi.org/10.3390/metabo2040775 

PMID: 24957762

• Deleterious effects of neuronal accumulation of glycogen in flies and mice.

Duran J, Tevy MF, Garcia-Rocha M, Calbó J, Milán M, Guinovart JJ.

EMBO Mol Med. 2012 Aug;4(8):719-29. doi: https://10.1002/emmm.201200241 

PMID: 22549942

• DOR/Tp53inp2 and Tp53inp1 constitute a metazoan gene family encoding dual regulators of autophagy and transcription.

Sancho A, Duran J, García-España A, Mauvezin C, Alemu EA, Lamark T, Macias MJ, DeSalle R, Royo M, Sala D, Chicote JU, Palacín M, Johansen T, Zorzano A.

PLoS One. 2012;7(3):e34034. doi: https://doi.org/10.1371/journal.pone.0034034 

PMID: 22470510

• Neurodegeneration and functional impairments associated with glycogen synthase accumulation in a mouse model of Lafora disease.

Valles-Ortega J*, Duran J*, Garcia-Rocha M, Bosch C, Saez I, Pujadas L, Serafin A, Cañas X, Soriano E, Delgado-García JM, Gruart A, Guinovart JJ.

EMBO Mol Med. 2011 Nov;3(11):667-81. doi: https://doi.org/10.1002/emmm.201100174 

PMID: 21882344

* co-authors

• The nuclear cofactor DOR regulates autophagy in mammalian and Drosophila cells.

Mauvezin C, Orpinell M, Francis VA, Mansilla F, Duran J, Ribas V, Palacín M, Boya P, Teleman AA, Zorzano A.

EMBO Rep. 2010 Jan;11(1):37-44. doi: https://doi.org/10.1038/embor.2009.242 

PMID: 20010805

• Identification of a novel modulator of thyroid hormone receptor-mediated action.

Baumgartner BG*, Orpinell M*, Duran J*, Ribas V, Burghardt HE, Bach D, Villar AV, Paz JC, González M, Camps M, Oriola J, Rivera F, Palacín M, Zorzano A.

PLoS One. 2007 Nov 21;2(11):e1183. doi: https://doi.org/10.1371/journal.pone.0001183 

PMID: 18030323

*co-authors