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Muscle-specific expression of LARGE restores neuromuscular transmission deficits in dystrophic LARGE(myd) mice.

Gumerson JD, Davis CS, Kabaeva ZT, Hayes JM, Brooks SV, Michele DE.

Hum Mol Genet. 2013 Feb 15;22(4):757-68. doi: 10.1093/hmg/dds483. Epub 2012 Dec 6.


Soleus muscle in glycosylation-deficient muscular dystrophy is protected from contraction-induced injury.

Gumerson JD, Kabaeva ZT, Davis CS, Faulkner JA, Michele DE.

Am J Physiol Cell Physiol. 2010 Dec;299(6):C1430-40. doi: 10.1152/ajpcell.00192.2010. Epub 2010 Sep 15.


Transgenic overexpression of LARGE induces α-dystroglycan hyperglycosylation in skeletal and cardiac muscle.

Brockington M, Torelli S, Sharp PS, Liu K, Cirak S, Brown SC, Wells DJ, Muntoni F.

PLoS One. 2010 Dec 28;5(12):e14434. doi: 10.1371/journal.pone.0014434.


Residual laminin-binding activity and enhanced dystroglycan glycosylation by LARGE in novel model mice to dystroglycanopathy.

Kanagawa M, Nishimoto A, Chiyonobu T, Takeda S, Miyagoe-Suzuki Y, Wang F, Fujikake N, Taniguchi M, Lu Z, Tachikawa M, Nagai Y, Tashiro F, Miyazaki J, Tajima Y, Takeda S, Endo T, Kobayashi K, Campbell KP, Toda T.

Hum Mol Genet. 2009 Feb 15;18(4):621-31. doi: 10.1093/hmg/ddn387. Epub 2008 Nov 18.


Adeno-associated virus-mediated overexpression of LARGE rescues α-dystroglycan function in dystrophic mice with mutations in the fukutin-related protein.

Vannoy CH, Xu L, Keramaris E, Lu P, Xiao X, Lu QL.

Hum Gene Ther Methods. 2014 Jun;25(3):187-96. doi: 10.1089/hgtb.2013.151. Epub 2014 May 2.


Overexpression of LARGE suppresses muscle regeneration via down-regulation of insulin-like growth factor 1 and aggravates muscular dystrophy in mice.

Saito F, Kanagawa M, Ikeda M, Hagiwara H, Masaki T, Ohkuma H, Katanosaka Y, Shimizu T, Sonoo M, Toda T, Matsumura K.

Hum Mol Genet. 2014 Sep 1;23(17):4543-58. doi: 10.1093/hmg/ddu168. Epub 2014 Apr 9.


Dystroglycan matrix receptor function in cardiac myocytes is important for limiting activity-induced myocardial damage.

Michele DE, Kabaeva Z, Davis SL, Weiss RM, Campbell KP.

Circ Res. 2009 Nov 6;105(10):984-93. doi: 10.1161/CIRCRESAHA.109.199489. Epub 2009 Sep 24.


Disruption of the mouse Large gene in the enr and myd mutants results in nerve, muscle, and neuromuscular junction defects.

Levedakou EN, Chen XJ, Soliven B, Popko B.

Mol Cell Neurosci. 2005 Apr;28(4):757-69.


Endogenous glucuronyltransferase activity of LARGE or LARGE2 required for functional modification of α-dystroglycan in cells and tissues.

Inamori K, Willer T, Hara Y, Venzke D, Anderson ME, Clarke NF, Guicheney P, Bönnemann CG, Moore SA, Campbell KP.

J Biol Chem. 2014 Oct 10;289(41):28138-48. doi: 10.1074/jbc.M114.597831. Epub 2014 Aug 19.


Mutant glycosyltransferase and altered glycosylation of alpha-dystroglycan in the myodystrophy mouse.

Grewal PK, Holzfeind PJ, Bittner RE, Hewitt JE.

Nat Genet. 2001 Jun;28(2):151-4.


Defects in glycosylation impair satellite stem cell function and niche composition in the muscles of the dystrophic Large(myd) mouse.

Ross J, Benn A, Jonuschies J, Boldrin L, Muntoni F, Hewitt JE, Brown SC, Morgan JE.

Stem Cells. 2012 Oct;30(10):2330-41. doi: 10.1002/stem.1197.


LARGE glycans on dystroglycan function as a tunable matrix scaffold to prevent dystrophy.

Goddeeris MM, Wu B, Venzke D, Yoshida-Moriguchi T, Saito F, Matsumura K, Moore SA, Campbell KP.

Nature. 2013 Nov 7;503(7474):136-40. doi: 10.1038/nature12605. Epub 2013 Oct 16.


Aberrant development of neuromuscular junctions in glycosylation-defective Large(myd) mice.

Herbst R, Iskratsch T, Unger E, Bittner RE.

Neuromuscul Disord. 2009 May;19(5):366-78. doi: 10.1016/j.nmd.2009.02.011. Epub 2009 Apr 5.


The sarcolemma in the Large(myd) mouse.

Reed PW, Mathews KD, Mills KA, Bloch RJ.

Muscle Nerve. 2004 Nov;30(5):585-95.


Post-translational maturation of dystroglycan is necessary for pikachurin binding and ribbon synaptic localization.

Kanagawa M, Omori Y, Sato S, Kobayashi K, Miyagoe-Suzuki Y, Takeda S, Endo T, Furukawa T, Toda T.

J Biol Chem. 2010 Oct 8;285(41):31208-16. doi: 10.1074/jbc.M110.116343. Epub 2010 Aug 3.


Inhibition of dystroglycan cleavage causes muscular dystrophy in transgenic mice.

Jayasinha V, Nguyen HH, Xia B, Kammesheidt A, Hoyte K, Martin PT.

Neuromuscul Disord. 2003 Jun;13(5):365-75.


Dystroglycan mediates homeostatic synaptic plasticity at GABAergic synapses.

Pribiag H, Peng H, Shah WA, Stellwagen D, Carbonetto S.

Proc Natl Acad Sci U S A. 2014 May 6;111(18):6810-5. doi: 10.1073/pnas.1321774111. Epub 2014 Apr 21.


Adeno-associated viral-mediated LARGE gene therapy rescues the muscular dystrophic phenotype in mouse models of dystroglycanopathy.

Yu M, He Y, Wang K, Zhang P, Zhang S, Hu H.

Hum Gene Ther. 2013 Mar;24(3):317-30. doi: 10.1089/hum.2012.084.

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