Limb-girdle muscular dystrophy types 2D and 2F (LGMD 2D and 2F) are autosomal recessive disorders caused by mutations in the alpha- and delta sarcoglycan genes, respectively, leading to severe muscle weakness and degeneration. The cause of the disease has been well characterized and a number of animal models are available for pre-clinical studies to test potential therapeutic interventions. To ...

To investigate mechanisms in the pathogenesis of cardiomyopathy associated with mutations of the dystrophin-glycoprotein complex, we analyzed genetically engineered mice deficient for either alpha-sarcoglycan (Sgca) or delta-sarcoglycan (Sgcd). We found that only Sgcd null mice developed cardiomyopathy with focal areas of necrosis as the histological hallmark in cardiac and skeletal muscle. Abs...

Membrane lesions play an early role in the pathogenesis of muscular dystrophy. Using a new albumin-targeted contrast agent (MS-325), sarcolemmal integrity of two animal models for muscular dystrophy was studied by MRI. Intravenously injected MS-325 does not enter skeletal muscle of normal mice. However, mdx and Sgca-null mutant mice, animal models for Duchenne and sarcoglycan-deficient limb-gir...

Background The pathophysiology of SSc involves a complex interplay between immune dysfunction, vasculopathy, and fibrosis. soluble guanylate cyclase (sGC) pathway has been shown to improve vascular endothelial function. We designed Phase II study test whether treatment vasculopathy with an sGCa could slow the progression fibrosis in patients early progressive vasculopathy. Objectives To design ...

BACKGROUND An observational study to examine whether thrombolytic therapy in stroke patients realizes better quality of life outcomes compared to patients without thrombolytic therapy one year after stroke. We also examined whether daily functioning, mental functioning and activities improved after thrombolytic treatment. METHODS A total of 88 stroke patients were interviewed at home one year...

Hyperspectral images (HSIs) generally contain tens or even hundreds of spectral segments within a specific frequency range. Due to the limitations and cost imaging sensors, HSIs often trade spatial resolution for finer band resolution. To compensate loss maintain balance between space spectrum, existing algorithms were used obtain excellent results. However, these could not fully mine coupling ...

In software engineering, we have identified and described the model correspondence problem. To Describe system architecture and artifacts uses models and diagrams. Models contains series of versions. To understand how versions correspondence are difficult. So, we designed a framework based on Search and Ammolite algorithms, which can cardinally finds the correspondence software models. Models a...