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Primary cilia are solitary signalling organelles projecting from the surface of most cell types. Although the ciliary membrane is continuous with the plasma membrane it exhibits a unique phospholipid composition, a feature essential for normal cilia formation and function. Recent studies have illustrated that distinct phosphoinositide lipid species localise to specific cilia subdomains, and have begun to build a 'phosphoinositide map' of the cilium. The abundance and localisation of phosphoinositides are tightly regulated by the opposing actions of lipid kinases and lipid phosphatases that have also been recently discovered at cilia. The critical role of phosphoinositides in cilia biology is highlighted by the devastating consequences of genetic defects in cilia-associated phosphoinositide regulatory enzymes leading to ciliopathy phenotypes in humans and experimental mouse and zebrafish models. Here we provide a general introduction to primary cilia and the roles phosphoinositides play in cilia biology. In addition to increasing our understanding of fundamental cilia biology, this rapidly expanding field may inform novel approaches to treat ciliopathy syndromes caused by deregulated phosphoinositide metabolism. In the setting of intracranial neoplasms, EWSR1-cAMP Response Element-Binding Protein (CREB) transcription factor family fusions have been described in myxoid mesenchymal tumors, extremely rare entities with a close histopathologic and immunologic resemblance to myxoid subtype angiomatoid fibrous histiocytomas (AFH). Controversy exists on whether these central nervous system lesions are a subtype of myxoid AFH or a completely separate entity, which entitles a distinct clinical behavior and, consequently, a different approach to management. Upon review of the literature, only 14 cases of intracranial tumors harboring an EWSR1-CREB family fusion were identified, with only 3 cases presenting in middle-aged adults, none of which reported an EWSR1-CREM fusion mutation. Significant variability in reported radiographic and histopathological characteristics, as well as in clinical outcomes, was noted. Their similarity with other soft tissue tumors, added to the scarce information on its clinical behavior, represents a great diagnostic and therapeutic challenge to the treating physician. We present a rare case of EWSR1-CREM mutated intracranial myxoid mesenchymal tumor/myxoid subtype AFH presenting as persistent headaches in a 36-yr-old woman with radiographic evidence of rapid growth and extensive vasogenic edema, for which she underwent surgical resection. This represents a unique case of EWSR1-CREM mutated intracranial myxoid mesenchymal tumor presenting in adulthood, with evidence of aggressive behavior. This represents a unique case of EWSR1-CREM mutated intracranial myxoid mesenchymal tumor presenting in adulthood, with evidence of aggressive behavior.Irreversible facial palsy, generally post-traumatic or postsurgical, can have devastating consequences for the patient from a functional, aesthetic, and psychological point of view. Among all of the reconstructive techniques, the hemihypoglossal-facial nerve transfer, which avoids the complete section of the hypoglossal nerve, is preferred by senior authors because of its excellent results and very low morbidity.1-5 This technique can be carried out in any neurosurgical center because it requires only basic instruments of microsurgery and a high-speed drill. However, detailed knowledge of the anatomy of the facial nerve in both its intrapetrosal and extracranial segments and of the hypoglossal nerve in its cervical segment is essential.1,6,7 Thus, previous practice in a cadaveric laboratory is recommended. The purpose of this video is to describe the technical nuances and key points of hemihypoglossal-facial nerve transfer. It was made using the surgical videos of 5 patients with a complete and irreversible facial paralysis who were operated using this technique in our institution between May and September 2019, all of whom consented to the procedure and to use for scientific purposes. The footages were edited, making a film in which the surgical technique is described in a stepwise fashion, emphasizing its most important features. To conclude, we would like to emphasize that the timing of surgery is of utmost importance and that this technique is both effective and reliable. Figures in the video at 0054 and 0135 are reprinted by permission from CCC Springer Nature, Acta Neurochirurgica, Treatment of complete facial palsy in adults comparative study between direct hemihypoglossal-facial neurorrhaphy, hemihipoglossal-facial neurorrhaphy with grafts, and massater to facial nerve transfer. https://www.selleckchem.com/products/ipi-549.html Socolovsky M, Martins RS, di Masi G, Bonilla G, Siqueira M, vol 158, 945-957, copyright 2016.The adoption and dissemination of evidence-based programs is predicated on multiple factors, including the degree to which key stakeholders are motivated to implement program best practices. The present study focuses on the development of indicators that capture motivations of teachers to adopt school wellness programming since personal motivations are central to achieving sustainable impacts in these settings. The study specifically describes the measurement development and validation of the Self-Regulations for Educators Questionnaire (SREQ), designed to measure educators' autonomous and controlled motivation for adopting evidence-based programming in their schools. A naturalistic design to study motivation to adopt aspects of NFL PLAY60 programming through the NFL PLAY60 FitnessGram Partnership Project was used. A total of 1,106 teachers completed the SREQ online. Internal validity was assessed through exploratory and confirmatory factor analysis, and predictive validity using structural equation modeling (SEM). The results supported the two-factor solution with separate items capturing aspects of autonomous and controlled motivation. Both factors had good internal reliability and the item-total correlation coefficients were above 0.40 for both factors. The results also supported the predictive validity as autonomous motivation positively predicted the level of overall engagement, teaching effectiveness, fitness testing reports sent home, and completion of fitness testing with students (p less then .05). Controlled motivation positively predicted whether the teachers conducted fitness testing and the engagement of Play 60 Challenge (p less then .05). Findings suggest that the SREQ displays a number of psychometric characteristics that make the instrument useful for examining motivation of providers to implement evidence-based best practices.
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