160 research outputs found

    Establishing Conversational Cooperation

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    Proceedings of the Seventh Annual Meeting of the Berkeley Linguistics Society (1981), pp. 178-19

    Hypoglossal Neuropathology and Respiratory Activity in Pompe Mice

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    Pompe disease is a lysosomal storage disorder associated with systemic deficiency of acid α-glucosidase (GAA). Respiratory-related problems in Pompe disease include hypoventilation and upper airway dysfunction. Although these problems have generally been attributed to muscular pathology, recent work has highlighted the potential role of central nervous system (CNS) neuropathology in Pompe motor deficiencies. We used a murine model of Pompe disease to test the hypothesis that systemic GAA deficiency is associated with hypoglossal (XII) motoneuron pathology and altered XII motor output during breathing. Brainstem tissue was harvested from adult Gaa−/− mice and the periodic acid Schiff method was used to examine neuronal glycogen accumulation. Semi-thin (2 μm) plastic sections showed widespread medullary neuropathology with extensive cytoplasmic glycogen accumulation in XII motoneuron soma. We next recorded efferent XII bursting in anesthetized and ventilated Gaa−/− and B6/129 mice both before and after bilateral vagotomy. The coefficient of variation of respiratory cycle duration was greater in Gaa−/− compared to B6/129 mice (p < 0.01). Vagotomy caused a robust increase in XII inspiratory burst amplitude in B6/129 mice (239 ± 44% baseline; p < 0.01) but had little impact on burst amplitude in Gaa−/− mice (130 ± 23% baseline; p > 0.05). We conclude that CNS GAA deficiency results in substantial glycogen accumulation in XII motoneuron cell bodies and altered XII motor output. Therapeutic strategies targeting the CNS may be required to fully correct respiratory-related deficits in Pompe disease

    One Big Family, One Big House: An In-Depth Look at Lincoln\u27s Clinton Elementary School

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    Lincoln’s Clinton neighborhood is a nondescript one in many ways, not unlike hundreds of other neighborhoods around the nation. Mature trees line streets with decades-old houses in varying states of repair. And the neighborhood elementary school, a stately, 1920s-era brick building, is right out of Central Casting. But within this very ordinariness is a story about a neighborhood where many families struggle with poverty, as they have for decades in this corner of Lincoln, and where the schoolhouse doors open to a refuge for some 400 children who collectively speak a dozen languages and rely on the teachers and staff for reading, writing and math―and sometimes coats, shoes and food. Clinton Elementary School, as one child says in a poster on the first-floor hallway wall, is like one big family in one big house. Six student reporters and two student photographers from a University of Nebraska-Lincoln College of Journalism and Mass Communications depth-reporting class explored Clinton’s world in a six-month project that took them to graffiti-sprinkled alleys a stone’s throw from Clinton School and into the controlled chaos of elementary classrooms where teachers routinely fall in love with the diverse parade of students―who love them in return. This publication is a product of their work and that of the eight editing and design students, under the guidance of UNL faculty member Nancy Anderson, who created the magazine during a summer school magazine editing class. But most important, it is the product of the patience and enthusiasm of the Clinton Elementary School staff, led by Principal Mona Manley, who welcomed us to the school, answered endless questions and showed by her example how dynamic leaders can make a difference. Student reporters spent the day at Clinton on March 1 and returned numerous times through the end of the school year. We agreed at the outset to identify by first name only most of the children in these stories. In addition to getting to know the school, the UNL student reporters also explored the Clinton neighborhood with Lincoln Action Program neighborhood organizer Shawn Ryba, who took students on a neighborhood walking tour, and Clinton Neighborhood Association President Maurice Baker, who welcomed class members to an association meeting. Readers will meet a wide variety of teachers, students, school administrators, parents, law enforcement officials and other community members, all of whom willingly shared their perspectives about the Clinton neighborhood and school. They opened our eyes to see beyond the ordinariness of a neighborhood and instead glimpse a world where families face financial strains and language barriers and many children don’t have enough to eat. But they also showed us a world where a unique group of grown-ups tries to fix what’s fixable and turn every day into a smile. It matters for the Clinton children. And for us all. (Note: a non-optimized version of the PDF -- 115MB -- is available below as an Additional file.
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