Charcot-Marie-Tooth disease (CMT) is the most commonly inherited neurological disorder with a prevalence of 1 in 2500 people worldwide. Patients suffer from degeneration of the peripheral nerves that control sensory information of the foot/leg and hand/arm. Multiple mutations in the neurofilament light polypeptide gene, NEFL, cause CMT2E. Previous studies in transfected cells showed that expression of disease-associated neurofilament light chain variants results in abnormal intermediate filament networks associated with defects in axonal transport. We have now generated knock-in mice with two different point mutations in Nefl: P8R that has been reported in multiple families with variable age of onset and N98S that has been described as an early-onset, sporadic mutation in multiple individuals. Nefl(P8R/+) and Nefl(P8R/P8R) mice were indistinguishable from Nefl(+/+) in terms of behavioral phenotype. In contrast, Nefl(N98S/+) mice had a noticeable tremor, and most animals showed a hindlimb clasping phenotype. Immunohistochemical analysis revealed multiple inclusions in the cell bodies and proximal axons of spinal cord neurons, disorganized processes in the cerebellum and abnormal processes in the cerebral cortex and pons. Abnormal processes were observed as early as post-natal day 7. Electron microscopic analysis of sciatic nerves showed a reduction in the number of neurofilaments, an increase in the number of microtubules and a decrease in the axonal diameters. The Nefl(N98S/+) mice provide an excellent model to study the pathogenesis of CMT2E and should prove useful for testing potential therapies.
Society on the Edge offers historians of social science and public policy a valuable 'bird's-eye view' of research into 'social problems' in the US since 1945 (p. 57). The volume features nine chapters written by historians of US social science, each focusing on a different area of research on 'social problems': Savina Balasubramanian and Charles Camic contribute a chapter on the family, Andrew Jewett on education, Alice O'Connor on poverty, Leah N. Gordon on discrimination, George C. Galster on 'the Black ghetto', Jean-Baptiste Fleury on crime, Nancy Campbell on addiction, Andrew Scull on mental illness and Joy Rohde on war. These chapters are introduced by a thought-provoking chapter by the volume's editors, Phillippe Fontaine and Jefferson D. Pooley.Each chapter addresses three central themes. First, they chart how the very 'problem status' of different social problems has changed over the course of the twentieth century and the role that social scientists have played in this process. Second, they map the shifting jurisdictions of the different social-science disciplines that have laid claimsometimes in collaboration, sometimes in competitionto different social problems since 1945. Third, they explore the changing fates of the different explanations and solutions that have emerged from this jurisdictional jockeying and from federal politics, policy making and public debate.Within the kaleidoscopic landscape of the political, cultural, economic and disciplinary transformations documented by the volume's contributors, the role of social scientists in defining social problems emerges as a co-productive one. Fontaine and Pooley argue convincingly that we should see social scientists as neither the prime drivers nor the passive bystanders of political change, but rather as participants in a process of 'mutual shaping that enmeshes social scientists in the politics of American social problems' (p. 57). The subsequent chapters illustrate the multiple ways this co-productive process has played out in each of the social-problem areas covered.The volume paints a vivid picture of the shifting fates of different social-scientific disciplines as their authority over certain social problems waxed and waned during the course of the twentieth century. Fontaine and Pooley advise against us seeing this process of as one of simple succession. Instead, they frame it as the result of a 'lopsided resonance' between certain facets of an always plural academic sphere and the always shifting tides of politics and public attitudes (p. 57). Certain forms of disciplinary expertise become
Problem Behavior Theory (PBT) is an influential psychosocial theory that has shaped-and continues to shapemuch research on adolescent development in the United States and abroad. It is the product of over a half-century of research conducted by psychologists-cum-behavioral scientists Lee and Richard Jessor. This article engages two
Catalyst is a year-long, bilingual (English/Spanish) fellowship program for high school students and their teachers who live in communities affected by the war on drugs (WoD) that is being waged across the Americas. This educational effort is a response to the social suffering caused by the WoD. Catalyst is working to forge transnational networks of solidarity and analysis among youth on the frontlines of the WoD and to ensure that their voices are heard by the growing drug-policy reform movement. In this field note, I argue that existing abstinence- and prevention-based programs fail to address the structural roots of the WoD and that a radical, more comprehensive approach to drug education is needed. I first lay out the context and rationale for the Catalyst program and then outline some of the challenges and lessons that emerged during its inaugural session. Based on facilitators' and students' experiences at that session, the program is seen as a promising first step toward an alternative approach to drug education. I conclude the field note by suggesting new avenues for inquiry and collaboration between the field of education in emergencies and drug-policy reform.
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