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 13 of 13 for “"Utrophin"”.
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A biochemical and molecular analysis of functional differences between dystrophin and utrophin
… aim at upregulating its autosomal homolog utrophin due to its structural similarity and ability to bind an almost identical repertoire of proteins that dystrophin binds. It was previously shown that utrophin cannot bind neuronal nitric oxide synthase (nNOS) even though dystrophin binds …
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Targeting transcriptional and translational mechanisms to enhance utrophin A expression as a therapy for Duchenne muscular dystrophy
… integrity and progressive muscle wasting. Utrophin is the autosomal homologue of dystrophin and is present in skeletal muscles of DMD patients, although its expression is primarily restricted to the neuromuscular junction of mature skeletal muscle fibers. Enhancing expression of utrophin in …
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Conformational changes in actinin-type actin binding domains: probing actin-induced structural dynamics in dystrophin and utrophin using EPR spectroscopy.
… Dystrophin, and its autosomal homologue utrophin, connect with the actin cytoskeleton through two F-actin binding domains, including an N-terminal "actinin-type" actin binding domain (ABD).In addition to dystrophin and utrophin, actinin-type ABDs are found in a large number of proteins. …
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The Effect of Resveratrol On Skeletal Muscle Function, Muscle Pathology, and Oxidative Capacity In the Mdx Mouse Model of Duchenne Muscular Dystrophy
… enhances oxidative metabolism, and activates the utrophin promoter; a gene whose protein product reduces dystrophic muscle damage. Therefore resveratrol treatment would likely benefit dystrophic muscle by reducing muscle pathology and increasing muscle function. The primary purpose of this study …
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Dissecting the signaling and mechanical functions of the dystrophin-glycoprotein complex in skeletal muscle
… affected mice deficient in both dystrophin and utrophin (a closely related homologue) prevented muscle wasting and increased survival. Dp116 did not restore the signaling molecule neuronal nitric oxide synthase (nNOS) to the DGC, and specific parts of the dystrophin rod domain not found in …
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An Alpha7beta1 Integrin-Based Treatment of Muscular Dystrophy
… mice lacking dystrophin and a related protein, utrophin. In mdx/utr-/- mice, a twofold increase in integrin was sufficient to reduce muscle degeneration, improve mobility, and extend longevity by threefold. The mechanism of rescue in transgenic mdx/utr-/- mice was independent of the …
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Identification and characterisation of novel marker proteins involved in X-linked muscular dystrophy
… a replacement of dystrophin with its homologue utrophin, restoration of β-dystroglycan was observed along with an increased concentration in heat shock proteins. While the severely affected cardiac muscle exhibited drastic decreases in the expression levels of many proteins involved in energy …
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Immune Response Markers are Prevalent in the mRNA Expression Profile of Maturing Dystrophic Murine Skeletal Muscle
… expression of mRNA in maturing dystrophin- and utrophin-deficient mouse (mdx:utrn-/-) muscles. Two potential dystrophic onset mechanisms targeted for analysis were (1) disrupted expression of calcium handling proteins; and, (2) increased expression of immune response markers. An mRNA expression …
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Mechanical Properties of Maturing Dystrophic Skeletal Muscle
… the muscle that only occurs with a lack of both utrophin and dystrophin. Fast-twitch and slow-twitch muscle mechanical properties were compared in control and dystrophic mice aged 3, 5, and 9 weeks of age. Dystrophic and control slow-twitch muscles did not have different mechanical properties, …
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Sarcoplasmic Reticulum Calcium Handling in Maturing Skeletal Muscle From Two Models of Dystrophic Mice
… mdx mouse which lacks dystrophin, and the mdx:utrophin-deficient (mdx:utrn<sup>-/-</sup>) mouse which also lacks utrophin, a protein homolog of dystrophin. The rate of SR Ca²⁺ uptake in quadriceps muscles of mdx/utrn<sup>-/-</sup> mice aged 21 days was 73.1% and 61.3% higher than age-matched …
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The Role of [beta]2-Syntrophin Phosphorylation in Secretory Granule Exocytosis
… in turn binds to the F-actin-binding protein utrophin. These data also indicate that stimulation of SG exocytosis affects the phosphorylation of b2-syntrophin,hence altering its binding to ICA512.Therefore a model was proposed whereby SGs are anchored to the actin cytoskeleton through the …
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Ca²⁺ and phosphoinositides regulations in α-actinin -4 F-actin binding.
… from other members of the spectrin superfamily, utrophin and dystrophin. Two of them reside in the CH1 domain and the third resides in the first α-helix of the CH2 domain. In addition, a PIP2 binding site has been mapped on a region adjacent to actin-binding site-3. These observations imply the …
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Cytotoxic T lymphocyte biology under the microscope
… bioprobes based on two truncations of utrophin, an actin-binding protein. These probes bind to actin filaments of the cytoskeleton in a manner proportional to the tension/compression of the filament, since tension affects filament torsion, which allows for ratiometric fluorescence …