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Enzymatic processing of beta-dystroglycan recombinant ectodomain by MMP-9: identification of the main cleavage siteGenetic Engineering of Dystroglycan in Animal Models of Muscular DystrophyRole of gelatinases in pathological and physiological processes involving the dystrophin-glycoprotein complexInsights from molecular dynamics simulations: structural basis for the V567D mutation-induced instability of zebrafish alpha-dystroglycan and comparison with the murine modelThe Structure of the T190M Mutant of Murine α-Dystroglycan at High Resolution: Insight into the Molecular Basis of a Primary DystroglycanopathyFirst genetic analysis of lattice corneal dystrophy type I in a family from Bulgaria.A synthetic peptide corresponding to the 550-585 region of alpha-dystroglycan binds beta-dystroglycan as revealed by NMR spectroscopy.Identification of the beta-dystroglycan binding epitope within the C-terminal region of alpha-dystroglycan.Proteasome Activity Is Affected by Fluctuations in Insulin-Degrading Enzyme Distribution.Functional diversity of dystroglycan.The multiple affinities of α-dystroglycan.Concerted mutation of Phe residues belonging to the beta-dystroglycan ectodomain strongly inhibits the interaction with alpha-dystroglycan in vitro.The structure of the N-terminal region of murine skeletal muscle alpha-dystroglycan discloses a modular architecture.A new monoclonal antibody DAG-6F4 against human alpha-dystroglycan reveals reduced core protein in some, but not all, dystroglycanopathy patients.Enzymatic processing by MMP-2 and MMP-9 of wild-type and mutated mouse β-dystroglycan.Evaluation of the effect of a floxed Neo cassette within the dystroglycan (Dag1) gene.A dystroglycan mutation (p.Cys667Phe) associated to muscle-eye-brain disease with multicystic leucodystrophy results in ER-retention of the mutant protein.Immunodetection of partially glycosylated isoforms of alpha-dystroglycan by a new monoclonal antibody against its beta-dystroglycan-binding epitope.Quantification, 2DE analysis and identification of enriched glycosylated proteins from mouse muscles: Difficulties and alternatives.A second Ig-like domain identified in dystroglycan by molecular modelling and dynamicsGenetic analysis of the dystroglycan gene in bronchopulmonary dysplasia affected premature newbornsStructural and functional analysis of the N-terminal extracellular region of beta-dystroglycanPlasticity of secondary structure in the N-terminal region of beta-dystroglycanActivation of muscle-specific receptor tyrosine kinase and binding to dystroglycan are regulated by alternative mRNA splicing of agrinalpha-Dystroglycan does not play a major pathogenic role in autosomal recessive hereditary inclusion-body myopathy
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description
onderzoeker
@nl
researcher ORCID ID = 0000-0002-2123-1516
@en
name
Francesca Sciandra
@ast
Francesca Sciandra
@en
Francesca Sciandra
@es
Francesca Sciandra
@nl
type
label
Francesca Sciandra
@ast
Francesca Sciandra
@en
Francesca Sciandra
@es
Francesca Sciandra
@nl
prefLabel
Francesca Sciandra
@ast
Francesca Sciandra
@en
Francesca Sciandra
@es
Francesca Sciandra
@nl
P106
P31
P496
0000-0002-2123-1516