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Article

Rewiring enervated: thinking LARGEr than myodystrophy.

Journal of neuroscience research - 1 Aug 2006

Levedakou Eleni N, Popko Brian

Abstract excerpt

LARGE is a glycosyltransferase known to glycosylate alpha-dystroglycan, a component of the dystrophin-associated glycoprotein complex. Spontaneous deletions in the Large gene (Large(myd) and Large(vls)) result in muscular dystrophy accompanied by heart, brain, and eye defects. Another Large mouse mutant, enervated (Large(enr)), is the result of a transgene integration event that disrupts Large gene expression. In...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

Identifiers

PMID: 16710847
DOI: 10.1002/jnr.20896

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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Rewiring enervated: thinking LARGEr than myodystrophy.PMID 16710847Read ↗

Investigating the functions of LARGE: lessons from mutant mice.Semantic proximity Read ↗

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

Mutant glycosyltransferase and altered glycosylation of alpha-dystroglycan in the myodystrophy mouse.Semantic proximity Read ↗

Muscle-specific expression of LARGE restores neuromuscular transmission deficits in dystrophic LARGEmyd miceSemantic proximity Read ↗

Transgenic Overexpression of LARGE Induces α-Dystroglycan Hyperglycosylation in Skeletal and Cardiac MuscleSemantic proximity Read ↗

Localization and functional analysis of the LARGE family of glycosyltransferases: significance for muscular dystrophy.Semantic proximity Read ↗

LARGE glycans on dystroglycan function as a tunable matrix scaffold to prevent dystrophy.Semantic proximity Read ↗

Mutation of Large, which encodes a putative glycosyltransferase, in an animal model of muscular dystrophy.Semantic proximity Read ↗

<i>Large1</i> Gene Transfer in Older <i>myd</i> Mice with Severe Muscular Dystrophy Restores Muscle Function and Greatly Improves SurvivalSemantic proximity Read ↗

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

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Intragenic deletion in the LARGE gene causes Walker-Warburg syndrome.Semantic proximity Read ↗

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