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Items: 1 to 20 of 150

1.

Glycogen metabolism in tissues from a mouse model of Lafora disease.

Wang W, Lohi H, Skurat AV, DePaoli-Roach AA, Minassian BA, Roach PJ.

Arch Biochem Biophys. 2007 Jan 15;457(2):264-9.

2.

Relationship between glycogen accumulation and the laforin dual specificity phosphatase.

Wang W, Parker GE, Skurat AV, Raben N, DePaoli-Roach AA, Roach PJ.

Biochem Biophys Res Commun. 2006 Nov 24;350(3):588-92.

3.

Laforin, the dual-phosphatase responsible for Lafora disease, interacts with R5 (PTG), a regulatory subunit of protein phosphatase-1 that enhances glycogen accumulation.

Fernández-Sánchez ME, Criado-García O, Heath KE, García-Fojeda B, Medraño-Fernández I, Gomez-Garre P, Sanz P, Serratosa JM, Rodríguez de Córdoba S.

Hum Mol Genet. 2003 Dec 1;12(23):3161-71.

PMID:
14532330
4.

Glycogen hyperphosphorylation underlies lafora body formation.

Turnbull J, Wang P, Girard JM, Ruggieri A, Wang TJ, Draginov AG, Kameka AP, Pencea N, Zhao X, Ackerley CA, Minassian BA.

Ann Neurol. 2010 Dec;68(6):925-33. doi: 10.1002/ana.22156.

PMID:
21077101
5.

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

Tagliabracci VS, Turnbull J, Wang W, Girard JM, Zhao X, Skurat AV, Delgado-Escueta AV, Minassian BA, Depaoli-Roach AA, Roach PJ.

Proc Natl Acad Sci U S A. 2007 Dec 4;104(49):19262-6.

6.

Abnormal metabolism of glycogen phosphate as a cause for Lafora disease.

Tagliabracci VS, Girard JM, Segvich D, Meyer C, Turnbull J, Zhao X, Minassian BA, Depaoli-Roach AA, Roach PJ.

J Biol Chem. 2008 Dec 5;283(49):33816-25. doi: 10.1074/jbc.M807428200.

7.

Genetic depletion of the malin E3 ubiquitin ligase in mice leads to lafora bodies and the accumulation of insoluble laforin.

DePaoli-Roach AA, Tagliabracci VS, Segvich DM, Meyer CM, Irimia JM, Roach PJ.

J Biol Chem. 2010 Aug 13;285(33):25372-81. doi: 10.1074/jbc.M110.148668.

8.

The phosphatase activity of laforin is dispensable to rescue Epm2a-/- mice from Lafora disease.

Gayarre J, Duran-Trío L, Criado Garcia O, Aguado C, Juana-López L, Crespo I, Knecht E, Bovolenta P, Rodríguez de Córdoba S.

Brain. 2014 Mar;137(Pt 3):806-18. doi: 10.1093/brain/awt353.

PMID:
24430976
9.

Novel glycogen synthase kinase 3 and ubiquitination pathways in progressive myoclonus epilepsy.

Lohi H, Ianzano L, Zhao XC, Chan EM, Turnbull J, Scherer SW, Ackerley CA, Minassian BA.

Hum Mol Genet. 2005 Sep 15;14(18):2727-36.

PMID:
16115820
10.

Laforin preferentially binds the neurotoxic starch-like polyglucosans, which form in its absence in progressive myoclonus epilepsy.

Chan EM, Ackerley CA, Lohi H, Ianzano L, Cortez MA, Shannon P, Scherer SW, Minassian BA.

Hum Mol Genet. 2004 Jun 1;13(11):1117-29.

PMID:
15102711
11.

Laforin prevents stress-induced polyglucosan body formation and Lafora disease progression in neurons.

Wang Y, Ma K, Wang P, Baba O, Zhang H, Parent JM, Zheng P, Liu Y, Minassian BA, Liu Y.

Mol Neurobiol. 2013 Aug;48(1):49-61. doi: 10.1007/s12035-013-8438-2.

12.

Muscle glycogen remodeling and glycogen phosphate metabolism following exhaustive exercise of wild type and laforin knockout mice.

Irimia JM, Tagliabracci VS, Meyer CM, Segvich DM, DePaoli-Roach AA, Roach PJ.

J Biol Chem. 2015 Sep 11;290(37):22686-98. doi: 10.1074/jbc.M115.673897.

13.

Laforin-malin complex degrades polyglucosan bodies in concert with glycogen debranching enzyme and brain isoform glycogen phosphorylase.

Liu Y, Zeng L, Ma K, Baba O, Zheng P, Liu Y, Wang Y.

Mol Neurobiol. 2014 Apr;49(2):645-57. doi: 10.1007/s12035-013-8546-z.

14.

The carbohydrate-binding domain of Lafora disease protein targets Lafora polyglucosan bodies.

Ganesh S, Tsurutani N, Suzuki T, Hoshii Y, Ishihara T, Delgado-Escueta AV, Yamakawa K.

Biochem Biophys Res Commun. 2004 Jan 23;313(4):1101-9.

PMID:
14706656
15.

Glycogen phosphomonoester distribution in mouse models of the progressive myoclonic epilepsy, Lafora disease.

DePaoli-Roach AA, Contreras CJ, Segvich DM, Heiss C, Ishihara M, Azadi P, Roach PJ.

J Biol Chem. 2015 Jan 9;290(2):841-50. doi: 10.1074/jbc.M114.607796.

16.

Laforin is required for the functional activation of malin in endoplasmic reticulum stress resistance in neuronal cells.

Zeng L, Wang Y, Baba O, Zheng P, Liu Y, Liu Y.

FEBS J. 2012 Jul;279(14):2467-78. doi: 10.1111/j.1742-4658.2012.08627.x.

17.

Glycogen accumulation underlies neurodegeneration and autophagy impairment in Lafora disease.

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

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

PMID:
24452334
18.

Advances in lafora progressive myoclonus epilepsy.

Delgado-Escueta AV.

Curr Neurol Neurosci Rep. 2007 Sep;7(5):428-33. Review.

PMID:
17764634
19.

Lafora progressive myoclonus epilepsy: NHLRC1 mutations affect glycogen metabolism.

Couarch P, Vernia S, Gourfinkel-An I, Lesca G, Gataullina S, Fedirko E, Trouillard O, Depienne C, Dulac O, Steschenko D, Leguern E, Sanz P, Baulac S.

J Mol Med (Berl). 2011 Sep;89(9):915-25. doi: 10.1007/s00109-011-0758-y.

20.

Increased endoplasmic reticulum stress and decreased proteasomal function in lafora disease models lacking the phosphatase laforin.

Vernia S, Rubio T, Heredia M, Rodríguez de Córdoba S, Sanz P.

PLoS One. 2009 Jun 16;4(6):e5907. doi: 10.1371/journal.pone.0005907.

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