Fukutin-related protein associates with the sarcolemmal dystrophin-glycoprotein complex
about
Fukutin-related protein resides in the Golgi cisternae of skeletal muscle fibres and forms disulfide-linked homodimers via an N-terminal interactionMouse fukutin deletion impairs dystroglycan processing and recapitulates muscular dystrophyMammalian O-mannosylation: unsolved questions of structure/functionBiomechanics of the sarcolemma and costameres in single skeletal muscle fibers from normal and dystrophin-null miceBasal lamina strengthens cell membrane integrity via the laminin G domain-binding motif of alpha-dystroglycanPOMK mutations disrupt muscle development leading to a spectrum of neuromuscular presentationsStructural basis of laminin binding to the LARGE glycans on dystroglycan.Efficacy of Gene Therapy Is Dependent on Disease Progression in Dystrophic Mice with Mutations in the FKRP GenePost-Natal knockdown of fukutin-related protein expression in muscle by long-termRNA interference induces dystrophic pathology [corrected]From proteins to genes: immunoanalysis in the diagnosis of muscular dystrophies.Abnormal Skeletal Muscle Regeneration plus Mild Alterations in Mature Fiber Type Specification in Fktn-Deficient Dystroglycanopathy Muscular Dystrophy Mice.Muscle protein alterations in LGMD2I patients with different mutations in the Fukutin-related protein geneFour-week rapamycin treatment improves muscular dystrophy in a fukutin-deficient mouse model of dystroglycanopathyMuscular dystrophies due to defective glycosylation of dystroglycan.Dystroglycan glycosylation and its role in alpha-dystroglycanopathiesMuscular dystrophies due to glycosylation defects.Congenital muscular dystrophy. Part II: a review of pathogenesis and therapeutic perspectives.Finding the sweet spot: assembly and glycosylation of the dystrophin-associated glycoprotein complex.AAV-mediated transfer of FKRP shows therapeutic efficacy in a murine model but requires control of gene expression.Mouse models of human ocular disease for translational research.Alterations of dystrophin-associated glycoproteins in the heart lacking dystrophin or dystrophin and utrophin.Autologous intramuscular transplantation of engineered satellite cells induces exosome-mediated systemic expression of Fukutin-related protein and rescues disease phenotype in a murine model of limb-girdle muscular dystrophy type 2I.Expression in retinal neurons of fukutin and FKRP, the protein products of two dystroglycanopathy-causative genes.
P2860
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P2860
Fukutin-related protein associates with the sarcolemmal dystrophin-glycoprotein complex
description
2007 թուականի Յունիսին հրատարակուած գիտական յօդուած
@hyw
2007 թվականի հունիսին հրատարակված գիտական հոդված
@hy
article publié dans la revue scientifique Journal of Biological Chemistry
@fr
artículu científicu espublizáu en 2007
@ast
im Juni 2007 veröffentlichter wissenschaftlicher Artikel
@de
scientific journal article
@en
vedecký článok (publikovaný 2007/06/08)
@sk
vědecký článek publikovaný v roce 2007
@cs
wetenschappelijk artikel (gepubliceerd op 2007/06/08)
@nl
наукова стаття, опублікована в червні 2007
@uk
name
Fukutin-related protein associates with the sarcolemmal dystrophin-glycoprotein complex
@ast
Fukutin-related protein associates with the sarcolemmal dystrophin-glycoprotein complex
@en
Fukutin-related protein associates with the sarcolemmal dystrophin-glycoprotein complex
@nl
type
label
Fukutin-related protein associates with the sarcolemmal dystrophin-glycoprotein complex
@ast
Fukutin-related protein associates with the sarcolemmal dystrophin-glycoprotein complex
@en
Fukutin-related protein associates with the sarcolemmal dystrophin-glycoprotein complex
@nl
prefLabel
Fukutin-related protein associates with the sarcolemmal dystrophin-glycoprotein complex
@ast
Fukutin-related protein associates with the sarcolemmal dystrophin-glycoprotein complex
@en
Fukutin-related protein associates with the sarcolemmal dystrophin-glycoprotein complex
@nl
P2093
P2860
P3181
P356
P1476
Fukutin-related protein associates with the sarcolemmal dystrophin-glycoprotein complex
@en
P2093
Aaron M. Beedle
Kevin P. Campbell
Patricia M. Nienaber
P2860
P304
16713–16717
P3181
P356
10.1074/JBC.C700061200
P407
P577
2007-06-08T00:00:00Z