Global ETD Search
Search theses and dissertations gathered from participating repositories worldwide. Every result links back to the library that holds it. No account is needed.
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Showing 1 to 9 of 9 for “"dystrophin glycoprotein complex"”.
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Dissecting the signaling and mechanical functions of the dystrophin-glycoprotein complex in skeletal muscle
… is caused by mutations in the gene encoding the dystrophin protein. Dystrophin is required for assembly of the dystrophin-glycoprotein complex (DGC) and provides a mechanically strong link between the cytoskeleton and the extracellular matrix. Several DGC proteins also participate in signaling …
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Protein-Protein Interactions and Muscle cell Signaling Via Syntrophin
<p>Absence of dystrophin results in Duchenne muscular dystrophy (DMD), a lethal neuromuscular d isorder that afflicts 1 in 3500 live male births. In the sarcolemma, dystrophin is associated with a complex of proteins and glycoproteins, known as the dystrophin glycoprotein complex. The DGC …
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Optimization of an in vitro model to study Duchenne Muscular Dystrophy
… from genetic and biochemical defects in the dystrophin-glycoprotein complex causing membrane instability, and accordingly, muscle fragility, apoptosis and abnormal calcium levels. To date, a clear understanding of the pathophysiology behind DMD remains elusive. Taking advantage of …
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The growth of murine breast cancer cells in dystrophic mice
… decreased quality of life. Alterations to the dystrophin glycoprotein complex (DGC), a transmembrane, multi-subunit protein complex with structural and signaling roles, have been reported in mammary tumors of BC patients and skeletal muscles of cachectic cancer patients. However, this complex …
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A biochemical and molecular analysis of functional differences between dystrophin and utrophin
The DMD gene encodes the protein dystrophin, a 427kD cytoplasmic protein responsible for linking the actin cytoskeleton to the extracellular matrix via the dystrophin-glycoprotein complex. Mutations in dystrophin that abolish its expression lead to Duchenne muscular dystrophy (DMD). Patients with …
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Fibromodulin and Dystrophin in Atherosclerosis: Novel roles for extracellular matrix in plaque development
… with the anti-inflammatory cytokine IL-10. Dystrophin, and the dystrophin-glycoprotein complex (DGC), link the actin cytoskeleton to the basement membrane. This thesis provides novel data on dystrophin deficiency, as illustrated by the mdx mouse. The absence of dystrophin stimulates …
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Increased structure-bound proteolytic activity in maturing dystrophic skeletal muscle
… the absence of the membrane-associated protein dystrophin and the secondary components of the dystrophin-glycoprotein complex. Although the genetic basis of the disease has been known for over 15 years, the onset mechanism of the disease is not yet known and no treatment is yet available to …
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CORRECTION OF THE GENETIC DEFECT IN INDUCED PLURIPOTENT STEM CELL LINES THROUGH CHROMOSOME TRANSPLANTATION
… model that resemble better the human anatomy and complexity compared to animal models across various applications. The coupling of iPSCs with advanced gene-editing technologies, such as CRISPR/Cas9, represents one of the most promising strategies for gene correction in a wide range of genetic …
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Chronic Dietary Supplementation of Branched-Chain Amino Acids Does Not Attenuate Muscle Torque Loss in a Mouse Model of Duchenne Muscular Dystrophy
… disease characterized by mutations in the dystrophin gene. Duchenne muscular dystrophy is the most common and most severe form of inherited muscle diseases, with an incidence of 1 in 3,500 male births1,2. Mutations in the dystrophin gene result in non-functional dystrophin or the complete …