4,679 research outputs found
The future looks brighter after 25 years of retinal gene therapy
No full textThe first report of in vivo gene delivery to the retina dates back to 1987 when a retroviral vector was injected intraocularly in newborn mice. Later came the observation that retinal cells could be successfully transduced using adenoviral and, then, adeno-associated and lentiviral vectors. By 2000, it had became clear that the eye, compared with other organs and tissues, provides a number of advantages for in vivo gene therapy with regard to safety, efficacy and route to clinical application. This has prompted the development of many successful proof-of-concept studies in animal models. The demonstration that sight could be restored in a large animal model with a congenital form of blindness was a major landmark that opened the door to the first-in-human trials for recessively inherited blinding conditions. With these first human studies demonstrating safety as well as some efficacy, retinal gene therapy has now come of age. Rapid clinical development has highlighted various new challenges, including the treatment of patients with advanced photoreceptor degeneration or dominantly inherited retinal dystrophies and those with defects in large genes, yet given the progress over the last 25 years, we expect a bright future for retinal gene therapy
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No full textThere is already a copy of this book in the collection. It is signed by the author. This copy is not signed. As I wrote there, this book represents a curious find. The Kalilah and Dimnah story is treated here, to the author's knowledge for the first time, as a play. The play seems talky; it contains plenty of aphorizing and a few fables. Still, this text includes music for three dances. And there are delightful colored illustrations: four early introduce the main personages, and sixteen with the English text show scenes along the way. Five black-and-white illustrations ornament the Arabic text that runs from the back to the middle. The colored illustrations present human form and clothes, animal faces, and Persian costume of the nineteenth century. Dimnah is pure Iago here, betraying both Lion and " Shatrebeh. " Leopard happens to overhear Kalilah upbraiding Dimnah after the fact; his testimony leads to Dimnah's punishment of imprisonment without food or water.There is already a copy of this book in the collection. It is signed by the author. This copy is not signed. As I wrote there, this book represents a curious find. The Kalilah and Dimnah story is treated here, to the author's knowledge for the first time, as a play. The play seems talky; it contains plenty of aphorizing and a few fables. Still, this text includes music for three dances. And there are delightful colored illustrations: four early introduce the main personages, and sixteen with the English text show scenes along the way. Five black-and-white illustrations ornament the Arabic text that runs from the back to the middle. The colored illustrations present human form and clothes, animal faces, and Persian costume of the nineteenth century. Dimnah is pure Iago here, betraying both Lion and " Shatrebeh. " Leopard happens to overhear Kalilah upbraiding Dimnah after the fact; his testimony leads to Dimnah's punishment of imprisonment without food or water.This is a hardbound book (hard cover)Language note: Bilingual: English/ArabicLanguage note: Bilingual: English/ArabicAli R. Amir-MoezAli R. Amir-Moe
Cervico-thoracic kyphosis in a girl with Pierre Robin sequence
No full textCongenital cervico-thoracic kyphosis has been encountered in a girl with Pierre Robin sequence. The constellation of the spine malformation complex such as incomplete development of the vertebral bodies associated with defective ossification of the cervico-thoracic pedicles causing effectively the development of complete spinal cord injury at the kyphotic level of C7/T1 were present. Congenital kyphosis secondary to vertebral body hypoplasia has not been reported in connection with Pierre Robin sequence
Weber-Type Integral Transform Connected with Robin-Type Boundary Conditions
No full textA new Weber-type integral transform and its inverse are defined for the representation of a function f(r,t), (r,t)∈[R,1]×[0,∞) that satisfies the Dirichlet and Robin-type boundary conditions f(R,t)=f1(t), f(1,t)−α∂f(r,t)∂r|r=1=f2(t), respectively. The orthogonality relations of the transform kernel are derived by using the properties of Bessel functions. The new Weber integral transform of some particular functions is determined. The integral transform defined in the present paper is a suitable tool for determining analytical solutions of transport problems with sliding phenomena that often occur in flows through micro channels, pipes or blood vessels. The heat conduction in an annular domain with Robin-type boundary conditions is studied. The subroutine “root(⋅)” of the Mathcad software is used to determine the positive roots of the transcendental equation involved in the definition of the new integral transform
An analytical study of the theatre of the Syrian playwright Saadallah Wannous, with particular emphasis on the plays written after the 1967 war
No full textThis study is an examination of the life and work of
the Syrian dramatist Saadallah Wannous (1941-1997). Wannous's name is virtually unknown in the West; only two academic studies of any significance have appeared in English on this eminent and challenging writer, who was honoured by UNESCO at the end of his life. Even in the Arab world his standing rests largely upon his celebrity as a cultural icon, since professional performances of his plays are rare due to the decline of the theatre in the region, and little attention has been devoted to theatre studies by Arab academics. The two studies in English do not attempt to be comprehensive but focus on particular stages of Wannous's career. This study is, therefore, the first to encompass the full range of Wannous's work. To do so it combines an account of his life which seeks to comprehend the various forces that shaped his thinking with an analysis of his dramatic works. The study concentrates on the plays written in the years following the trauma inflicted on the Arab world by the catastrophe of their
defeat in the Arab-Israeli war of June, 1967. Wannous's career can be divided into three phases: the immature plays of his young manhood which are influenced by European models and generally focus on the social condition of the individual; his middle period - the `theatre of politicisation', when his Marxist politics were the main
factor shaping his drama; and his late works, which are characterised by an extraordinary freedom of thought and expression. The introduction places Wannous in his historical and sociocultural context and provides a brief background explaining the literary and theatrical traditions of the Arab world that influenced his activity as a dramatist. Each phase is then examined in turn and the plays are analysed in accordance with the focus of the study. This means that emphasis is given to the middle period,
but no significant work is neglected. The study aims to trace the trajectory of Wannous's development using a
variety of sources: the plays themselves, Wannous's own journalism and critical writings, interviews with his widow, his friends and colleagues, and numerous
journals, books and articles, some of which contain
important interviews with Wannous that shed light on his thought and ways of working. Use is also made of the
two studies mentioned above. The study shows that Wannous's theatre was influenced by the key political, social and cultural developments of his time, and that he
constantly sought to find forms that would express those transformations in dramatic terms
Family altruism and incentives
No full textThe author builds on the altruistic model of the family, to explore the strategic interaction between altruistic parents, and selfish children, when children's efforts are endogenous. If there is uncertainty about the amount of income the children will realize, and if parents have imperfect information, the children have an incentive to exert little effort, and to rely on their parent's altruistically motivated transfers. Because of this, parents face a tradeoff between the insurance that bequests implicitly provide their children, and the disincentive to work prompted by their altruism. The author shows that if parents can credibly commit to a pattern of transfers, they will choose not to compensate children in bad outcomes, as much as predicted by the standard (no uncertainty, no asymmetric information) dynastic model of the family. Alternatively, parents may choose to forgo any insurance, and offer a fixed level of bequest, to elicit greater effort from their children. The optimal transfers structure that the author derives, reconciles the predictions of the altruistic family model, with much of the existing evidence on inter-generational transfers, which suggests that parents compensate only partially, or not at all, for earnings differentials among their children. Moreover, the author shows that Ricardian equivalence holds in this setup, except when non-negativity constraints are binding.Economic Theory&Research,Environmental Economics&Policies,Health Economics&Finance,Educational Sciences,Safety Nets and Transfers
Adult ciliary epithelial cells, previously identified as retinal stem cells with potential for retinal repair, fail to differentiate into new rod photoreceptors
No full textThe ciliary margin in lower vertebrates is a site of continual retinal neurogenesis and a stem cell niche. By contrast, the human eye ceases retinal neuron production before birth and loss of photoreceptors during life is permanent and a major cause of blindness. The discovery of a proliferative cell population in the ciliary epithelium (CE) of the adult mammalian eye, designated retinal stem cells, raised the possibility that these cells could help to restore sight by replacing lost photoreceptors. We previously demonstrated the feasibility of photoreceptor transplantation using cells from the developing retina. CE cells could provide a renewable source of photoreceptors for transplantation. Several laboratories reported that these cells generate new photoreceptors, whereas a recent report questioned the existence of retinal stem cells. We used Nrl.gfp transgenic mice that express green fluorescent protein in rod photoreceptors to assess definitively the ability of CE cells to generate new photoreceptors. We report that CE cells expanded in monolayer cultures, lose pigmentation, and express a subset of eye field and retinal progenitor cell markers. Simultaneously, they continue to express some markers characteristic of differentiated CE and typically lack a neuronal morphology. Previously reported photoreceptor differentiation conditions used for CE cells, as well as conditions used to differentiate embryonic retinal progenitor cells (RPCs) and embryonic stem cell-derived RPCs, do not effectively activate the Nrl-regulated photoreceptor differentiation program. Therefore, we conclude that CE cells lack potential for photoreceptor differentiation and would require reprogramming to be useful as a source of new photoreceptors
Total Factor Productivity Growth and Agricultural Research and Extension: An Analysis of Pakistan's Agriculture, 1960-1996
No full textPakistan’s agriculture has grown rapidly since the 1960s, with an average annual growth of about 4 percent over the four decades till the end of the century. Agricultural growth at this rate was sustained by the technological progress embodied in the high-yielding varieties of grains and cotton, with supporting public investment in irrigation, agricultural research and extension (R&E), and physical infrastructure. This rate of agricultural growth has significantly contributed to the overall economic growth of about 6 percent per year during this period. Sustaining this performance presents a considerable challenge for the public policy framework for agriculture, not the least for the agricultural research and extension system in Pakistan. This study analyses the impact of R&E investment on TFP growth in Pakistan’s agriculture within a distributed lag framework. The estimation of the productivity-R&E relationship with an Almon lag provided evidence of a strong relationship, explaining 96 percent of the variation in the TFP index. The marginal internal rate of return on R&E investment is estimated at 88 percent. This rate of return may look unusually high but it is well within the range of returns estimated in the context of developing and developed countries. The high rate of return is an indicator not only of under-investment in R&E but also of the constraints on research and extension services that prevent optimal performance
Cultural aspects of multi-channel customer management: A case study in Italy
No full textChannel management is one of CRM systems component much influenced by the behaviour of customers in relation to its implementation and use. The consumers’ behaviours, preferences, perceptions and expectations are crucial for the implementation and use of channel management. Customers’ contact with the organization’s multi-channels can occur at several touch points throughout the customer lifecycle. Customers’ behaviours may be differentiated according to the individual or micro level, but it might also differ at an ecological or macro level of analysis (Ramaseshan et al., 2006). In this paper the author has conducted a case study in a multinational organization in Italy to analyze customers’ behaviours at a macro level and customers channel choices, through out the customer lifecycle. The author has used a content analysis technique to define the themes of the case study and then used the Structurational Analysis model by Ali and Brooks, (2008) to identify the cultural values dimensions (Ali, et al. 2008a) that influence multi-channel customer management in Italy. The research findings highlight the cultural dimensions, which should be considered while adopting multi-channel customer strategy. Also, the research findings encourage the articulation of situated cultural approach to study cultural impact within IS discipline as alternative approach than predefined culture archetype
Negar Habibi. « ‘Ali Qoli Jebadar et l’enregisrement du réel dans les peintures dites farangi sazi »
No full textThis article is a mainly iconographic study of five paintings that were signed by or attributed to ‘Ali Qoli Jebadar. This so-called farangi sazi- artist was active at the Safavid court of Shah ‘Abbas II (r. 1642-1666) and Shah Soleyman (r. 1666-1694). With this study the author wants to demonstrate that ‘Ali Qoli Jebadar observed and “registered” the reality of daily life at the Safavid court, rather than depicting an ideal, timeless world. She does this by confronting certain subjects, obje..
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