Dystroglycan: from biosynthesis to pathogenesis of human disease
R Barresi, KP Campbell - Journal of cell science, 2006 - journals.biologists.com
α-and β-dystroglycan constitute a membrane-spanning complex that connects the
extracellular matrix to the cytoskeleton. Although a structural role for dystroglycan had been …
extracellular matrix to the cytoskeleton. Although a structural role for dystroglycan had been …
Laminin isoforms in endothelial and perivascular basement membranes
LF Yousif, J Di Russo, L Sorokin - Cell adhesion & migration, 2013 - Taylor & Francis
Laminins, one of the major functional components of basement membranes, are found
underlying endothelium, and encasing pericytes and smooth muscle cells in the vessel wall …
underlying endothelium, and encasing pericytes and smooth muscle cells in the vessel wall …
The dystrophin–glycoprotein complex, cellular signaling, and the regulation of cell survival in the muscular dystrophies
TA Rando - Muscle & nerve, 2001 - Wiley Online Library
Mutations of different components of the dystrophin–glycoprotein complex (DGC) cause
muscular dystrophies that vary in terms of severity, age of onset, and selective involvement …
muscular dystrophies that vary in terms of severity, age of onset, and selective involvement …
Muscular dystrophies involving the dystrophin–glycoprotein complex: an overview of current mouse models
M Durbeej, KP Campbell - Current opinion in genetics & development, 2002 - Elsevier
The dystrophin–glycoprotein complex (DGC) is a multisubunit complex that connects the
cytoskeleton of a muscle fiber to its surrounding extracellular matrix. Mutations in the DGC …
cytoskeleton of a muscle fiber to its surrounding extracellular matrix. Mutations in the DGC …
Dystroglycan Is Essential for Early Embryonic Development: Disruption of Reichert's Membrane in Dag1-Null Mice
RA Williamson, MD Henry, KJ Daniels… - Human molecular …, 1997 - academic.oup.com
Dystroglycan is a central component of the dystrophin-glycoprotein complex (DGC), a
protein assembly that plays a critical role in a variety of muscular dystrophies. In order to …
protein assembly that plays a critical role in a variety of muscular dystrophies. In order to …
[HTML][HTML] Dystrophin complex functions as a scaffold for signalling proteins
B Constantin - Biochimica et Biophysica Acta (BBA)-Biomembranes, 2014 - Elsevier
Dystrophin is a 427 kDa sub-membrane cytoskeletal protein, associated with the inner
surface membrane and incorporated in a large macromolecular complex of proteins, the …
surface membrane and incorporated in a large macromolecular complex of proteins, the …
Mutations in GDP-mannose pyrophosphorylase B cause congenital and limb-girdle muscular dystrophies associated with hypoglycosylation of α-dystroglycan
Congenital muscular dystrophies with hypoglycosylation of α-dystroglycan (α-DG) are a
heterogeneous group of disorders often associated with brain and eye defects in addition to …
heterogeneous group of disorders often associated with brain and eye defects in addition to …
The role of laminins in basement membrane function
M Aumailley, N Smyth - The Journal of Anatomy, 1998 - cambridge.org
Laminins are a family of multifunctional macromolecules, ubiquitous in basement
membranes, and represent the most abundant structural noncollagenous glycoproteins of …
membranes, and represent the most abundant structural noncollagenous glycoproteins of …
A role for dystroglycan in basement membrane assembly
MD Henry, KP Campbell - Cell, 1998 - cell.com
Basement membranes are composed of ordered arrays of characteristic extracellular matrix
proteins, but little is known about the assembly of these structures in vivo. We have …
proteins, but little is known about the assembly of these structures in vivo. We have …
Molecular and functional defects in kidneys of mice lacking collagen alpha 3 (IV): implications for Alport syndrome.
Collagen IV is a major structural component of all basal laminae (BLs). Six collagen IV alpha
chains are present in mammals; alpha 1 and alpha 2 (IV) are broadly expressed in embryos …
chains are present in mammals; alpha 1 and alpha 2 (IV) are broadly expressed in embryos …