[HTML][HTML] Structure and function of the dystrophin-glycoprotein complex

JM Ervasti - Madame curie bioscience database [Internet], 2013 - ncbi.nlm.nih.gov
Duchenne muscular dystrophy (DMD) is the most prevalent and severe form of human
muscular dystrophy. While clinical descriptions of DMD date back to the 1850's, over 100 …

Functions of dystrophin

SC Brown, JA Lucy - Dystrophin Gene, Protein and Cell Biology, 1997 - books.google.com
The absence of dystrophin in skeletal muscle is the underlying cause of Duchenne muscular
dystrophy (DMD), a severe form of muscle disease characterized by progressive muscle …

Function and genetics of dystrophin and dystrophin-related proteins in muscle

DJ Blake, A Weir, SE Newey… - Physiological …, 2002 - journals.physiology.org
The X-linked muscle-wasting disease Duchenne muscular dystrophy is caused by mutations
in the gene encoding dystrophin. There is currently no effective treatment for the disease; …

[HTML][HTML] Dystrophin, its interactions with other proteins, and implications for muscular dystrophy

JM Ervasti - Biochimica et Biophysica Acta (BBA)-Molecular Basis …, 2007 - Elsevier
Duchenne muscular dystrophy is the most prevalent and severe form of human muscular
dystrophy. Investigations into the molecular basis for Duchenne muscular dystrophy were …

Role of dystrophin isoforms and associated proteins in muscular dystrophy

KG Culligan, AJ Mackey, DM Finn… - International …, 1998 - spandidos-publications.com
The membrane cytoskeletal component dystrophin and its associated glycoproteins play a
central role in the molecular pathogenesis of several muscular dystrophies, ie …

Expression of dystrophin-associated proteins in dystrophin-positive muscle fibers (revertants) in Duchenne muscular dystrophy

K Matsumura, FMS Tome, H Collin, F Leturcq… - Neuromuscular …, 1994 - Elsevier
The dystrophin-glycoprotein complex spans the sarcolemma to provide a linkage between
the subsarcolemmal cytoskeleton and the extracellular matrix in skeletal muscle. In …

Phosphorylation within the cysteine-rich region of dystrophin enhances its association with β-dystroglycan and identifies a potential novel therapeutic target for …

K Swiderski, SA Shaffer, B Gallis… - Human Molecular …, 2014 - academic.oup.com
Mutations in dystrophin lead to Duchenne muscular dystrophy, which is among the most
common human genetic disorders. Dystrophin nucleates assembly of the dystrophin …

[PDF][PDF] Three muscular dystrophies: review loss of cytoskeleton-extracellular matrix linkage

KP Campbell - Cell, 1995 - Citeseer
Muscular dystrophies are a group of diseases that primarily affect skeletal muscle and are
characterized by progressive muscle wasting and weakness. Although these diseases have …

Proteomic profiling of the dystrophin complex and membrane fraction from dystrophic mdx muscle reveals decreases in the cytolinker desmoglein and increases in …

S Murphy, H Brinkmeier, M Krautwald, M Henry… - Journal of Muscle …, 2017 - Springer
The almost complete loss of the membrane cytoskeletal protein dystrophin and concomitant
drastic reduction in dystrophin-associated glycoproteins are the underlying mechanisms of …

Coiled-coil regions in the carboxy-terminal domains of dystrophin and related proteins: potentials for protein-protein interactions

DJ Blake, JM Tinsley, KE Davies… - Trends in biochemical …, 1995 - orca.cardiff.ac.uk
Dystrophin, the protein product of the DMD gene, is a component of the muscle-membrane
cytoskeleton. Mutations in the DMD gene result in the allelic myopathies Duchenne and …