LARGE glycans on dystroglycan function as a tunable matrix scaffold to prevent dystrophy
about
B4GAT1 is the priming enzyme for the LARGE-dependent functional glycosylation of α-dystroglycanThe glucuronyltransferase B4GAT1 is required for initiation of LARGE-mediated α-dystroglycan functional glycosylationGlycan Engineering for Cell and Developmental BiologyA POGLUT1 mutation causes a muscular dystrophy with reduced Notch signaling and satellite cell loss.POMK mutations disrupt muscle development leading to a spectrum of neuromuscular presentationsStructural basis of laminin binding to the LARGE glycans on dystroglycan.Dystroglycan mediates homeostatic synaptic plasticity at GABAergic synapsesPhosphorylation within the cysteine-rich region of dystrophin enhances its association with β-dystroglycan and identifies a potential novel therapeutic target for skeletal muscle wasting.Fukutin is prerequisite to ameliorate muscular dystrophic phenotype by myofiber-selective LARGE expression.Abnormal Skeletal Muscle Regeneration plus Mild Alterations in Mature Fiber Type Specification in Fktn-Deficient Dystroglycanopathy Muscular Dystrophy Mice.Transgenic Rescue of the LARGEmyd Mouse: A LARGE Therapeutic Window?Progressive Dystrophic Pathology in Diaphragm and Impairment of Cardiac Function in FKRP P448L Mutant Mice.Protein O-Mannosylation in the Murine Brain: Occurrence of Mono-O-Mannosyl Glycans and Identification of New SubstratesISPD produces CDP-ribitol used by FKTN and FKRP to transfer ribitol phosphate onto α-dystroglycan.Four-week rapamycin treatment improves muscular dystrophy in a fukutin-deficient mouse model of dystroglycanopathyLassa Virus Cell Entry via Dystroglycan Involves an Unusual Pathway of Macropinocytosis.Carbohydrate-binding domain of the POMGnT1 stem region modulates O-mannosylation sites of α-dystroglycanThrombospondin expression in myofibers stabilizes muscle membranes.Annexin A6 modifies muscular dystrophy by mediating sarcolemmal repair.Stress-induced ECM alteration modulates cellular microRNAs that feedback to readjust the extracellular environment and cell behavior.Mammalian O-mannosylation pathway: glycan structures, enzymes, and protein substrates.Finding the sweet spot: assembly and glycosylation of the dystrophin-associated glycoprotein complex.Glycobiology of α-dystroglycan and muscular dystrophy.Matriglycan: a novel polysaccharide that links dystroglycan to the basement membrane.Cardiac Physiology of Aging: Extracellular Considerations.Prenatal muscle development in a mouse model for the secondary dystroglycanopathiesAAV-mediated transfer of FKRP shows therapeutic efficacy in a murine model but requires control of gene expression.Glycan susceptibility factors in autism spectrum disorders.Overexpression of LARGE suppresses muscle regeneration via down-regulation of insulin-like growth factor 1 and aggravates muscular dystrophy in mice.Lassa Virus Cell Entry Reveals New Aspects of Virus-Host Cell InteractionProtein O-mannosylation is crucial for human mesencyhmal stem cells fate.Studies of Lassa Virus Cell Entry.Axl can serve as entry factor for Lassa virus depending on the functional glycosylation of dystroglycan.
P2860
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P2860
LARGE glycans on dystroglycan function as a tunable matrix scaffold to prevent dystrophy
description
2013 թուականի Նոյեմբերին հրատարակուած գիտական յօդուած
@hyw
2013 թվականի նոյեմբերին հրատարակված գիտական հոդված
@hy
article publié dans la revue scientifique Nature
@fr
artículu científicu espublizáu en 2013
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scientific journal article
@en
vedecký článok (publikovaný 2013/11/07)
@sk
vědecký článek publikovaný v roce 2013
@cs
wetenschappelijk artikel (gepubliceerd op 2013/11/07)
@nl
наукова стаття, опублікована в листопаді 2013
@uk
مقالة علمية (نشرت في 7-11-2013)
@ar
name
LARGE glycans on dystroglycan function as a tunable matrix scaffold to prevent dystrophy
@ast
LARGE glycans on dystroglycan function as a tunable matrix scaffold to prevent dystrophy
@en
LARGE glycans on dystroglycan function as a tunable matrix scaffold to prevent dystrophy
@nl
type
label
LARGE glycans on dystroglycan function as a tunable matrix scaffold to prevent dystrophy
@ast
LARGE glycans on dystroglycan function as a tunable matrix scaffold to prevent dystrophy
@en
LARGE glycans on dystroglycan function as a tunable matrix scaffold to prevent dystrophy
@nl
prefLabel
LARGE glycans on dystroglycan function as a tunable matrix scaffold to prevent dystrophy
@ast
LARGE glycans on dystroglycan function as a tunable matrix scaffold to prevent dystrophy
@en
LARGE glycans on dystroglycan function as a tunable matrix scaffold to prevent dystrophy
@nl
P2093
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P3181
P356
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LARGE glycans on dystroglycan function as a tunable matrix scaffold to prevent dystrophy
@en
P2093
David Venzke
Fumiaki Saito
Kiichiro Matsumura
Matthew M Goddeeris
Takako Yoshida-Moriguchi
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P2888
P304
P3181
P356
10.1038/NATURE12605
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P577
2013-10-16T00:00:00Z
P5875
P6179
1026295173