排序方式: 共有35条查询结果,搜索用时 406 毫秒
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Elisa Bianchini Stefania Testoni Arcangelo Gentile Tito Calì Denis Ottolini Antonello Villa Marisa Brini Romeo Betto Francesco Mascarello Poul Nissen Dorianna Sandonà Roberta Sacchetto 《The Journal of biological chemistry》2014,289(48):33073-33082
A missense mutation in ATP2A1 gene, encoding sarco(endo)plasmic reticulum Ca2+-ATPase (SERCA1) protein, causes Chianina cattle congenital pseudomyotonia, an exercise-induced impairment of muscle relaxation. Skeletal muscles of affected cattle are characterized by a selective reduction of SERCA1 in sarcoplasmic reticulum membranes. In this study, we provide evidence that the ubiquitin proteasome system is involved in the reduced density of mutated SERCA1. The treatment with MG132, an inhibitor of ubiquitin proteasome system, rescues the expression level and membrane localization of the SERCA1 mutant in a heterologous cellular model. Cells co-transfected with the Ca2+-sensitive probe aequorin show that the rescued SERCA1 mutant exhibits the same ability of wild type to maintain Ca2+ homeostasis within cells. These data have been confirmed by those obtained ex vivo on adult skeletal muscle fibers from a biopsy from a pseudomyotonia-affected subject. Our data show that the mutation generates a protein most likely corrupted in proper folding but not in catalytic activity. Rescue of mutated SERCA1 to sarcoplasmic reticulum membrane can re-establish resting cytosolic Ca2+ concentration and prevent the appearance of pathological signs of cattle pseudomyotonia. 相似文献
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Paola Pinti Clarisse Aichelburg Frida Lind Sarah Power Elizabeth Swingler Arcangelo Merla Antonia Hamilton Sam Gilbert Paul Burgess Ilias Tachtsidis 《Journal of visualized experiments : JoVE》2015,(106)
Functional Near Infrared Spectroscopy (fNIRS) is a neuroimaging technique that uses near-infrared light to monitor brain activity. Based on neurovascular coupling, fNIRS is able to measure the haemoglobin concentration changes secondary to neuronal activity. Compared to other neuroimaging techniques, fNIRS represents a good compromise in terms of spatial and temporal resolution. Moreover, it is portable, lightweight, less sensitive to motion artifacts and does not impose significant physical restraints. It is therefore appropriate to monitor a wide range of cognitive tasks (e.g., auditory, gait analysis, social interaction) and different age populations (e.g., new-borns, adults, elderly people). The recent development of fiberless fNIRS devices has opened the way to new applications in neuroscience research. This represents a unique opportunity to study functional activity during real-world tests, which can be more sensitive and accurate in assessing cognitive function and dysfunction than lab-based tests. This study explored the use of fiberless fNIRS to monitor brain activity during a real-world prospective memory task. This protocol is performed outside the lab and brain haemoglobin concentration changes are continuously measured over the prefrontal cortex while the subject walks around in order to accomplish several different tasks. 相似文献
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Maria-Angela Losi Stefano Nistri Maurizio Galderisi Sandro Betocchi Franco Cecchi Iacopo Olivotto Eustachio Agricola Piercarlo Ballo Simona Buralli Antonello D'Andrea Arcangelo D'Errico Donato Mele Susanna Sciomer Sergio Mondillo 《Cardiovascular ultrasound》2010,8(1):1-19
Hypertrophic cardiomyopathy (HCM) is one of the most common inherited cardiomyopathy. The identification of patients with HCM is sometimes still a challenge. Moreover, the pathophysiology of the disease is complex because of left ventricular hyper-contractile state, diastolic dysfunction, ischemia and obstruction which can be coexistent in the same patient. In this review, we discuss the current and emerging echocardiographic methodology that can help physicians in the correct diagnostic and pathophysiological assessment of patients with HCM. 相似文献
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