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252 related items for PubMed ID: 27088557

  • 1. Crystal structure of glycogen debranching enzyme and insights into its catalysis and disease-causing mutations.
    Zhai L, Feng L, Xia L, Yin H, Xiang S.
    Nat Commun; 2016 Apr 18; 7():11229. PubMed ID: 27088557
    [Abstract] [Full Text] [Related]

  • 2. Crystal structures of glycogen-debranching enzyme mutants in complex with oligosaccharides.
    Shen M, Gong X, Xiang S.
    Acta Crystallogr F Struct Biol Commun; 2021 Nov 01; 77(Pt 11):420-426. PubMed ID: 34726181
    [Abstract] [Full Text] [Related]

  • 3. Mutations in exon 3 of the glycogen debranching enzyme gene are associated with glycogen storage disease type III that is differentially expressed in liver and muscle.
    Shen J, Bao Y, Liu HM, Lee P, Leonard JV, Chen YT.
    J Clin Invest; 1996 Jul 15; 98(2):352-7. PubMed ID: 8755644
    [Abstract] [Full Text] [Related]

  • 4. Distinct mutations in the glycogen debranching enzyme found in glycogen storage disease type III lead to impairment in diverse cellular functions.
    Cheng A, Zhang M, Okubo M, Omichi K, Saltiel AR.
    Hum Mol Genet; 2009 Jun 01; 18(11):2045-52. PubMed ID: 19299494
    [Abstract] [Full Text] [Related]

  • 5. Association of bi-functional activity in the N-terminal domain of glycogen debranching enzyme.
    Min-Ho L, Hyung-Nam S, Ji-Eun C, Lan TP, Sunghoon P, Jong-Tae P, Eui-Jeon W.
    Biochem Biophys Res Commun; 2014 Feb 28; 445(1):107-12. PubMed ID: 24491554
    [Abstract] [Full Text] [Related]

  • 6. Structural rationale for the short branched substrate specificity of the glycogen debranching enzyme GlgX.
    Song HN, Jung TY, Park JT, Park BC, Myung PK, Boos W, Woo EJ, Park KH.
    Proteins; 2010 Jun 28; 78(8):1847-55. PubMed ID: 20187119
    [Abstract] [Full Text] [Related]

  • 7. New approach to prepare fluorogenic branched dextrins for assaying glycogen debranching enzyme.
    Sakaguchi M, Makino Y, Matsubara H.
    Glycoconj J; 2020 Dec 28; 37(6):667-679. PubMed ID: 33201379
    [Abstract] [Full Text] [Related]

  • 8. Rescue of GSDIII Phenotype with Gene Transfer Requires Liver- and Muscle-Targeted GDE Expression.
    Vidal P, Pagliarani S, Colella P, Costa Verdera H, Jauze L, Gjorgjieva M, Puzzo F, Marmier S, Collaud F, Simon Sola M, Charles S, Lucchiari S, van Wittenberghe L, Vignaud A, Gjata B, Richard I, Laforet P, Malfatti E, Mithieux G, Rajas F, Comi GP, Ronzitti G, Mingozzi F.
    Mol Ther; 2018 Mar 07; 26(3):890-901. PubMed ID: 29396266
    [Abstract] [Full Text] [Related]

  • 9. A Functional Human Glycogen Debranching Enzyme Encoded by a Synthetic Gene: Its Implications for Glycogen Storage Disease Type III Management.
    Triggiani D, Demurtas OC, Illiano E, Massa S, Pasquo A, Dionisi-Vici C, Marino C, Giuliano G, Franconi R.
    Protein Pept Lett; 2024 Mar 07; 31(7):519-531. PubMed ID: 39021187
    [Abstract] [Full Text] [Related]

  • 10. A functional mini-GDE transgene corrects impairment in models of glycogen storage disease type III.
    Gardin A, Rouillon J, Montalvo-Romeral V, Rossiaud L, Vidal P, Launay R, Vie M, Krimi Benchekroun Y, Cosette J, Bertin B, La Bella T, Dubreuil G, Nozi J, Jauze L, Fragnoud R, Daniele N, Van Wittenberghe L, Esque J, André I, Nissan X, Hoch L, Ronzitti G.
    J Clin Invest; 2024 Jan 16; 134(2):. PubMed ID: 38015640
    [Abstract] [Full Text] [Related]

  • 11. A nonsense mutation due to a single base insertion in the 3'-coding region of glycogen debranching enzyme gene associated with a severe phenotype in a patient with glycogen storage disease type IIIa.
    Shen J, Bao Y, Chen YT.
    Hum Mutat; 1997 Jan 16; 9(1):37-40. PubMed ID: 8990006
    [Abstract] [Full Text] [Related]

  • 12. [Diagnosis of glycogen storage disease type IIIA by detecting glycogen debranching enzyme activity, glycogen content and structure in muscle].
    Wang W, We M, Song HM, Qiu ZQ, Zhang WM, Wu XY, Lu CX, Qi JM, Jing H, Li F.
    Zhonghua Er Ke Za Zhi; 2009 Aug 16; 47(8):608-12. PubMed ID: 19951495
    [Abstract] [Full Text] [Related]

  • 13. Mutational analysis of the AGL gene: five novel mutations in GSD III patients.
    Lucchiari S, Donati MA, Melis D, Filocamo M, Parini R, Bresolin N, Comi GP.
    Hum Mutat; 2003 Oct 16; 22(4):337. PubMed ID: 12955720
    [Abstract] [Full Text] [Related]

  • 14. Donor substrate specificity of 4-alpha-glucanotransferase of porcine liver glycogen debranching enzyme and complementary action to glycogen phosphorylase on debranching.
    Watanabe Y, Makino Y, Omichi K.
    J Biochem; 2008 Mar 16; 143(3):435-40. PubMed ID: 18174188
    [Abstract] [Full Text] [Related]

  • 15. Acceptor specificity of 4-alpha-glucanotransferases of mammalian glycogen debranching enzymes.
    Makino Y, Omichi K.
    J Biochem; 2006 Mar 16; 139(3):535-41. PubMed ID: 16567418
    [Abstract] [Full Text] [Related]

  • 16. Expression of a Functional Recombinant Human Glycogen Debranching Enzyme (hGDE) in N. benthamiana Plants and in Hairy Root Cultures.
    Rodriguez-Hernandez M, Triggiani D, Ivison F, Demurtas OC, Illiano E, Marino C, Franconi R, Massa S.
    Protein Pept Lett; 2020 Mar 16; 27(2):145-157. PubMed ID: 31622193
    [Abstract] [Full Text] [Related]

  • 17. Glycogen debranching enzyme association with beta-subunit regulates AMP-activated protein kinase activity.
    Sakoda H, Fujishiro M, Fujio J, Shojima N, Ogihara T, Kushiyama A, Fukushima Y, Anai M, Ono H, Kikuchi M, Horike N, Viana AY, Uchijima Y, Kurihara H, Asano T.
    Am J Physiol Endocrinol Metab; 2005 Sep 16; 289(3):E474-81. PubMed ID: 15886229
    [Abstract] [Full Text] [Related]

  • 18. The Saccharomyces cerevisiae YPR184w gene encodes the glycogen debranching enzyme.
    Teste MA, Enjalbert B, Parrou JL, François JM.
    FEMS Microbiol Lett; 2000 Dec 01; 193(1):105-10. PubMed ID: 11094287
    [Abstract] [Full Text] [Related]

  • 19. Identification of the catalytic residues of bifunctional glycogen debranching enzyme.
    Nakayama A, Yamamoto K, Tabata S.
    J Biol Chem; 2001 Aug 03; 276(31):28824-8. PubMed ID: 11375985
    [Abstract] [Full Text] [Related]

  • 20. Crystal structure of Thermotoga maritima 4-alpha-glucanotransferase and its acarbose complex: implications for substrate specificity and catalysis.
    Roujeinikova A, Raasch C, Sedelnikova S, Liebl W, Rice DW.
    J Mol Biol; 2002 Aug 02; 321(1):149-62. PubMed ID: 12139940
    [Abstract] [Full Text] [Related]

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