The PROM1 mutation p.R373C causes an autosomal dominant bull's eye maculopathy associated with rod, rod-cone, and macular dystrophy.

PURPOSE: To characterize in detail the phenotype of five unrelated families with autosomal dominant bull's eye maculopathy (BEM) due to the R373C mutation in the PROM1 gene. METHODS: Forty-one individuals of five families of Caribbean (family A), British (families B, D, E), and Italian (family C) origin, segregating the R373C mutation in PROM1, were ascertained. Electrophysiological assessment, fundus autofluorescence (FAF) imaging, fundus fluorescein angiography (FFA), and optical coherence tomography (OCT) were performed in available subjects. Mutation screening of PROM1 was performed. RESULTS: The R373C mutant was present heterozygously in all affected patients. The age at onset was variable and ranged between 9 and 58 years, with most of the individuals presenting with reading difficulties. Subjects commonly had a mild to moderate reduction in visual acuity except for members of family C who experienced markedly reduced central vision. The retinal phenotype was characterized by macular dystrophy, with retinal pigment epithelial mottling in younger subjects, progressing to typical BEM over time, with the development of macular atrophy in older patients. In addition, all members of family C had typical features of RP. The electrophysiological findings were variable both within and between families. CONCLUSIONS: Mutations in PROM1 have been described to cause a severe form of autosomal recessive RP in two families of Indian and Pakistani descent. The results of this study have demonstrated that a distinct redundant PROM1 mutation (R373C) can also produce an autosomal dominant, fully penetrant retinopathy, characterized by BEM with little inter- and intrafamilial variability, and retinal dystrophy with variable rod or rod-cone dysfunction and marked intra- and interfamilial variability, ranging from isolated maculopathy without generalized photoreceptor dysfunction to maculopathy associated with very severe rod-cone dysfunction.

Addressing elementary school teachers' professional stressors: Practical suggestions for schools and adminstrators

Purpose: Given the preponderance of education reform since the No Child Left Behind Act (U.S. Department of Education, 2001), reform efforts have shaped the nature of the work and culture in schools. The emphasis on standardized testing to determine schools' status and student performance, among other factors, has generated stress, particularly for teachers. Therefore, district and school administrators are encouraged to consider the contextual factors that contribute to teacher stress to address them and to retain high-performing teachers. Research Methods/Approach: Participants were recruited from two types of schools in order to test hypotheses related to directional responding as a function of working in a more challenging (high-priority) or less challenging (non-high-priority) school environment. We employed content analysis to analyze 64 suburban elementary school teachers' free-responses to a prompt regarding their stress as teachers. We cross-analyzed our findings through external auditing to bolster trustworthiness in the data and in the procedure. Findings: Teachers reported personal and contextual stressors. Herein, we reported concrete examples of the five categories of contextual stressors teachers identified: political and educational structures, instructional factors, student factors, parent and family factors, and school climate. We found directional qualities and overlapping relationships in the data, partially confirming our hypotheses. Implications for Research and Practice: We offer specific recommendations for practical ways in which school administrators might systemically address teacher stress based on the five categories of stressors reported by participants. We also suggest means of conducting action research to measure the effects of implemented suggestions.

Reading, Translating, Rewriting: Angela Carter's Translational Poetics

In "Reading, Translating, Rewriting: Angela Carter's Translational Poetics", author Martine Hennard Dutheil de la Rochère delves into Carter's The Fairy Tales of Charles Perrault (1977) to illustrate that this translation project had a significant impact on Carter's own writing practice. Hennard combines close analyses of both texts with an attention to Carter's active role in the translation and composition process to explore this previously unstudied aspect of Carter's work. She further uncovers the role of female fairy-tale writers and folktales associated with the Grimms' Kinder- und Hausmärchen in the rewriting process, unlocking new doors to The Bloody Chamber. Hennard begins by considering the editorial evolution of The Fairy Tales of Charles Perrault from 1977 to the present day, as Perrault's tales have been rediscovered and repurposed. In the chapters that follow, she examines specific linkages between Carter's Perrault translation and The Bloody Chamber, including targeted analysis of the stories of Red Riding Hood, Bluebeard, Puss-in-Boots, Beauty and the Beast, Sleeping Beauty, and Cinderella. Hennard demonstrates how, even before The Bloody Chamber, Carter intervened in the fairy-tale debate of the late 1970s by reclaiming Perrault for feminist readers when she discovered that the morals of his worldly tales lent themselves to her own materialist and feminist goals. Hennard argues that The Bloody Chamber can therefore be seen as the continuation of and counterpoint to The Fairy Tales of Charles Perrault, as it explores the potential of the familiar stories for alternative retellings. While the critical consensus reads into Carter an imperative to subvert classic fairy tales, the book shows that Carter valued in Perrault a practical educator as well as a proto-folklorist and went on to respond to more hidden aspects of his texts in her rewritings. Reading, Translating, Rewriting is informative reading for students and teachers of fairy-tale studies and translation studies.

Rising to Greatness An Imperative for Improving Iowa’s Schools, 2011

In the early 1990s, the Cold War ended, Back to the Future III was in theaters, and Iowa led the nation in reading and mathematics. Times have changed. A decade into the 21st century, Iowa has conceded its place at the top. During the past 20 years, achievement trends illustrate Iowa’s slide from a national leader in PK-12 education to a national average―sometimes below average―performer as other states (and nations) have accelerated past the state. Iowa students’ futures are at risk. Collectively, Iowa students are not hitting the mark in mathematics and reading competency. Sure, Iowa has its share of super-achievers. But the mass of Iowa students—not just underprivileged or minority students, but many of the majority white, relatively affluent students as well—are falling short of what is needed to attain quality jobs, growing incomes, and secure livelihoods in today’s globally competitive world. The world has moved beyond the industrial age and information age and is now in the innovation age. Students must be armed not only with knowledge, but also with skills and insights needed to critically analyze and innovate. The pressing problems and grand opportunities the world faces require that many more people contribute as innovators and problem solvers, not order takers and implementers. Innovators will prosper. Order takers will stagnate. The days of an abundance of low-skill jobs have come to an end.

Reading films through political classics : genres of politics in popular cultures

Abstract: Readin films through political classics

One Unshakable Vision: World Class Schools for Iowa, October 3, 2011

Iowans have long shared a deep commitment to giving our children the best education possible. We recognize young people today must meet higher expectations than ever to thrive in this global, knowledge-based economy. For the sake of our children and our state, it is vitally important that we build on our tradition of excellence to improve our schools. Iowa’s house of education still has a strong foundation, but it is also in need of a major remodel to be ready for the days ahead.

Some critical reflections on Michel Foucault's reading of Plutarch's Eroticus (Amatorius)

This aim of this article is to reflect on Michel Foucault's reading of Plutarch's Eroticus in his Histoire de la sexualité, putting emphasis on the fact that, against what it is affirmed by the French thinker, the real debate is not, in the author's opinion, about true pleasure, that obtained by the erastés from his erómenos or that obtained by husbands from their wives, but the need to assign also love and friendship (éros kaì philia) to the conjugal love.

Leaving the cave in search of life (A Platonic reading of R. Sirera's La caverna (The Cave)

The aim of this article is to show how a contemporary playwright thinks once more of the Platonic image of the cave in order to reflect on the necessary existential journey of men and women as in the case of a Bildungsroman. Sooner or later men and women must abandon the protection that any sort of cavern such as home, the family garden or family itself can offer. In spite of writing from a by no means idealistic or metaphysical point of view, thanks to R. Sirera and to the very applicability of Platonic images, Plato becomes once again a classical reference which is both useful and even unavoidable if one bears in mind the Platonic origin of all the literary caverns.

Patriarchal Hauntings: Re-reading Villainy and Gender in Daphne du Maurier's Rebecca

Criticism of Daphne du Maurier’s popular classic Rebecca (1938) can be divided into two mainstream interpretations. On the one hand, it was traditionally marketed as a gothic romance where the hero and the heroine conquered the evil women that separated them. On the other, certain feminist critics have recently provided a very diff erent view of the story, aligning it with gothic narratives that deal with the dangers women suff er under the patriarchal control of their husbands. Th is would imply that du Maurier’s novel should not be read as a traditional romance that promotes patriarchy, as the former interpretation suggested. In this article, I propose, through a Gothic Studies and a Gender Studies reading, that villainy in this novel is not exclusively linked to gender and, therefore, the victim and abuser statuses cannot be equated to femininity and masculinity, respectively. Instead, I argue that villainy in the novel is inextricably connected to being in a powerful position within patriarchy, and that it is the desire to maintain this position and perpetuate the patriarchal system that leads the main characters (men and women) to commit acts of villainy.