Diseases causing inherited retinal degeneration in humans, such as retinitis pigmentosa and macular dystrophy, are genetically heterogeneous and clinically diverse. More than 40 genes causing retinal degeneration have been mapped to specific chromosomal sites; of these, at least 10 have been cloned and characterized. Mutations in two proteins, rhodopsin and peripherin/RDS, account for approximately 35% of all cases of autosomal dominant retinitis pigmentosa and a lesser fraction of other retinal conditions. This target article reviews the genes and mutations causing retinal (...) degeneration and proposes mechanisms whereby specific mutations lead to particular clinical consequences, that is, the relationship between genotype and phenotype. Retinitis pigmentosa and macular dystrophy are genetically heterogeneous diseases that cause retinal degeneration in humans and often result in severe visual impairment or blindness. Although many of the genes causing these diseases have not been identified, three photoreceptor-specific proteins have been implicated: rhodopsin, peripherin/RDS, and the P-subunit of rod phosphodiesterase. Mutations in the genes for these three proteins can cause either dominant retinitis pigmentosa, recessive retinitis pigmentosa, dominant congenital stationary night blindness, or dominant macular degeneration. Why this multiplicity of clinical phenotypes? Our target article summarizes the genetic and biochemical background to this question and proposes a number of possible explanations. Discussion focuses mainly on 73 distinct disease-causing mutations of rhodopsin. We feel that rhodopsin and other photoreceptor proteins can serve as model systems for unraveling the connection between genotype and phenotype, not only for inherited retinal diseases but for other degenerative disorders as well. (shrink)

Recent findings emphasize the complexity, both genetic and functional, of the manifold genes and mutations causing inherited retinal degeneration in humans. Knowledge of the genetic bases of these diseases can contribute to design of rational therapy, as well as elucidating the function of each gene product in normal visual processes.

This chapter contains sections titled: What is a Father? Fatherly Mercy as Attitude Mercy Enables Connection with Children Mercy Strengthens Relationships What Does Fatherly Mercy Look Like? Notes.

What is the importance of deconstruction, and the writing of Jacques Derrida in particular, for literary criticism today? Derek Attridge argues that the challenge of Derrida's work for our understanding of literature and its value has still not been fully met, and in this book, which traces a close engagement with Derrida's writing over two decades and reflects an interest in that work going back a further two decades, shows how that work can illuminate a variety of topics. Chapters include (...) an overview of deconstruction as a critical practice today, discussions of the secret, postcolonialism, ethics, literary criticism, jargon, fiction, and photography, and responses to the theoretical writing of Emmanuel Levinas, Roland Barthes, and J. Hillis Miller. Also included is a discussion of the recent reading of Derrida's philosophy as 'radical atheism', and the book ends with a conversation on deconstruction and place with the theorist and critic Jean-Michel Rabate. Running throughout is a concern with the question of responsibility, as exemplified in Derrida's own readings of literary and philosophical texts: responsibility to the work being read, responsibility to the protocols of rational argument, and responsibility to the reader. (shrink)

It is widely assumed that disability is typically a bad thing for those who are disabled. Our purpose in this essay is to critique this view and defend a more nuanced picture of the relationship between disability and well-being. We first examine four interpretations of the above view and argue that it is false on each interpretation. We then ask whether disability is thereby a neutral trait. Our view is that most disabilities are neutral in one sense, though we cannot (...) make simple generalizations about disability's relationship to well-being in other important senses. After defending this view, we discuss its practical implications for selective abortion for disability, nondisabled people's interactions with disabled people, and the use of QALYs in health policy. (shrink)

What makes some human rights campaigns for the physical integrity rights of prisoners more effective than others? Despite various normative arguments condemning these practices, only limited systematic analysis documents the relative effectiveness of different arguments on individuals. This is surprising, because the success of human rights campaigns depends on getting individuals to care about and support policy positions that protect human rights. We constructed an experiment to compare the effects of six different arguments against prisoner abuse and torture. We found (...) that an argument which emphasized the suffering of the prisoner had a consistently positive and significant effect on opposition to torture and prisoner abuse. However, this effect was largely contingent on subjects’ political ideology. Political conservatives actually became less opposed to torture, on average, after reading the same argument emphasizing the prisoners’ suffering or the sacredness of human beings. (shrink)

It may seem obvious that causing disability in another person is morally problematic in a way that removing or preventing a disability is not. This suggests that there is a moral asymmetry between causing disability and causing non-disability. This chapter investigates whether there are any differences between these two types of actions that might explain the existence of a general moral asymmetry. After setting aside the possibility that having a disability is almost always bad or harmful for a person (a (...) view that we have critiqued at length elsewhere), seven putative differences are considered. Ultimately, it is concluded that none of these seven factors can ground a general moral asymmetry between causing disability and causing non-disability, though each factor can provide some moral reason to avoid causing disability in certain particular cases. (shrink)

It is commonly thought that disability is a harm or “bad difference” because having a disability restricts valuable options in life. In his recent essay “Disability, Options and Well-Being,” Thomas Crawley offers a novel defense of this style of reasoning and argues that we and like-minded critics of this brand of argument are guilty of an inconsistency. Our aim in this article is to explain why our view avoids inconsistency, to challenge Crawley's positive defense of the Options Argument, and to (...) suggest that this general line of reasoning employs a double standard. (shrink)

(Anna Maria Taigi 1769 - 1837)St. Alphonsus writes: "a single bad book will be sufficient to cause the destruction of a monastery." Pope Pius XII wrote in 1947 at the beatification of Blessed Maria Goretti: "There rises to Our lips the cry of the Saviour: 'Woe to the world because of scandals!' (Matthew 18:7). Woe to those who consciously and deliberately spread corruption-in novels, newspapers, magazines, theaters, films, in a world of immodesty!" We at St. Pius X Press are calling (...) for a crusade of good books. We want to restore 1,000 old Catholic books to the market. We ask for your assistance and prayers. This book is a photographic reprint of the original. The original has been inspected and many imperfections in the existing copy have been corrected. At Saint Pius X Press our goal is to remain faithful to the original in both photographic reproductions and in textual reproductions that are reprinted. Photographic reproductions are given a page by page inspection, whereas textual reproductions are proofread to correct any errors in reproduction. (shrink)

How does the visual system recognize images of a novel object after a single observation despite possible variations in the viewpoint of that object relative to the observer? One possibility is comparing the image with a prototype for invariance over a relevant transformation set. However, invariance over rotations has proven difficult to analyze, because it applies to some objects but not others. We propose that the invariant transformations of an object are learned by incorporating prior expectations with real-world evidence. We (...) test this proposal by developing an ideal learner model for learning invariance that predicts better learning of orientation dependence when prior expectations about orientation are weak. This prediction was supported in two behavioral experiments, where participants learned the orientation dependence of novel images using feedback from solving arithmetic problems. (shrink)

Donald Trump has been a godsend for those of us who teach critical thinking. For he is a fount of manipulative rhetoric, glaring fallacies, conspiracy theories, fake news, and bullshit. In this paper I draw on my own recent teaching experience in order to discuss both the usefulness and the limits of using Trump examples in teaching critical thinking. In Section One I give the framework of the course; in Section Two I indicate Trump’s relevance to many important concepts in (...) the course; and in Section Three I argue that critical thinking instructors should restrain themselves from overreliance on Trump-examples. (shrink)

Atheist philosophers Jean-Paul Sartre and Kurt Baier, though from different philosophical traditions, shared a common concern about the traditional Judeo-Christian-Muslim doctrine that human beings are the creations of a Supreme Being. For Sartre, in “Existentialism is a Humanism” (1946), a God who designed us would thereby detract from our freedom and dignity. For Baier, in “The Meaning of Life” (1957), the idea that God designs us to serve his own purposes was deeply offensive in treating us as artifacts, domestic animals, (...) or slaves. Indeed, Baier said explicitly what was implicit in Sartre: that the divine creation of humans as would violate Immanuel Kant’s respect for persons principle. But this Kantian objection is badly flawed in ignoring the crucial role of human free will in traditional Abrahamic theism. Still, if we focus not on the divine-creation doctrine but on the doctrine of eternal torment for non-worshippers of God, then traditional Abrahamic theism does arguably undermine human freedom and dignity. For the threat of eternal torment is extraordinarily coercive. Keywords: theism, artifacts, free will, eternal torment, coercion. (shrink)

In his 1967 paper 'A Causal Theory of Knowing', Alvin Goldman sketched an account of empirical knowledge in terms of appropriate causal connections between the fact known and the knower's belief in that fact. This early causal account has been much criticized, even by Goldman himself in later years. We argue that the theory is much more defensible than either he or its other critics have recognized, that there are plausible internal and external resources available to it which save it (...) from many objections in the literature (in particular, objections raised by Harman, Pappas and Swain, Klein, Dretske, Goldman, Shope, Ackermann, Morawetz, and Collier). (shrink)

The Free Discussion between Richard Price and Joseph Priestley (1778) originated as a correspondence between the two after the publication of Priestley's Disquisitions on Matter and Spirit, his most important philosophical work (1777). At the time it was thought remarkable that a controversey such as this could be conducted so amicably, but then the two were close friends. Nevertheless their philosophical, as opposed to their oft mentioned political views, were at opposite ends of a spectrum.

This paper describes the design of a control and management network (orderwire) for a mobile wireless Asynchronous Transfer Mode (ATM) network. This mobile wireless ATM network is part of the Rapidly Deployable Radio Network (RDRN). The orderwire system consists of a packet radio network which overlays the mobile wireless ATM network, each network element in this network uses Global Positioning System (GPS) information to control a beamforming antenna subsystem which provides for spatial reuse. This paper also proposes a novel Virtual (...) Network Configuration (VNC) algorithm for predictive network configuration. A mobile ATM Private Network-Network Interface (PNNI) based on VNC is also discussed. Finally, as a prelude to the system implementation, results of a Maisie simulation of the orderwire system are discussed. (shrink)

With increased frequency, reproductive technologies are placing prospective parents in the position of choosing whether to bring a disabled child into the world. The most well-known objection to the act of “selecting against disability” is known as the Expressivist Argument. The argument claims that such acts express a negative or disrespectful message about disabled people and that one has a moral reason to avoid sending such messages. We have two primary aims in this essay. The first is to critically examine (...) the standard Expressivist Argument, which we analyze in terms of the expression of ableist attitudes. We distinguish three interpretations of the argument and argue that each version faces serious objections. Our second aim is to articulate two closely related arguments that also pertain to expressed ableist attitudes. The Expressive Harms Argument maintains that there is a moral reason to avoid actions that give rise to the perception of ableist attitudes in cases where this is likely to have negative consequences for disabled people. The Ableist Motivation Argument speaks against selecting against disability in cases where one would be motivated by ableist attitudes. While these two arguments face various objections, we seek to establish that they are more promising than the most natural interpretations of the Expressivist Argument. (shrink)

This work has been selected by scholars as being culturally important and is part of the knowledge base of civilization as we know it. This work is in the public domain in the United States of America, and possibly other nations. Within the United States, you may freely copy and distribute this work, as no entity (individual or corporate) has a copyright on the body of the work. Scholars believe, and we concur, that this work is important enough to be (...) preserved, reproduced, and made generally available to the public. To ensure a quality reading experience, this work has been proofread and republished using a format that seamlessly blends the original graphical elements with text in an easy-to-read typeface. We appreciate your support of the preservation process, and thank you for being an important part of keeping this knowledge alive and relevant. (shrink)

Screening for asymptomatic disease is a routine aspect of contemporary public health practice. However, it is also controversial, because it leads to overdiagnosis and overtreatment, with many arguing that programmes are “ineffective,” i.e., the “costs” outweigh the “benefits.” This paper explores a more fundamental objection to screening programmes: that, even if they are effective, they are ethically impermissible because they breach the principle of non-maleficence. In so doing, it suggests a new approach to the ethics of risk, justifying a concern (...) with how policies affect individuals’ absolute ex-ante prospects. Part 1 sets up the tension between screening and non-maleficence. Part 2 introduces and motivates a novel interpretation of the non-maleficence principle, “ex-ante Do No Harm,” which resolves this tension. Part 3 defends and clarifies this principle by discussing its relationship to the ex-ante Pareto principle. Part 4 discusses the worry that risk estimates are too “subjective.”. (shrink)

There is a trend toward the use of predictive systems in communications networks. At the systems and network management level predictive capabilities are focused on anticipating network faults and performance degradation. Simultaneously, mobile communication networks are being developed with predictive location and tracking mechanisms. The interactions and synergies between these systems present a new set of problems. A new predictive network management framework is developed and examined. The interaction between a predictive mobile network and the proposed network management system is (...) discussed. The Rapidly Deployable Radio Network is used as a specific example to illustrate these interactions. (shrink)

In his 1967 paper 'A Causal Theory of Knowing', Alvin Goldman sketched an account of empirical knowledge in terms of appropriate causal connections between the fact known and the knower's belief in that fact. This early causal account has been much criticized, even by Goldman himself in later years. We argue that the theory is much more defensible than either he or its other critics have recognized, that there are plausible internal and external resources available to it which save it (...) from many objections in the literature (in particular, objections raised by Harman, Pappas and Swain, Klein, Dretske, Goldman, Shope, Ackermann, Morawetz, and Collier). (shrink)

That people do wrong would appear to be a moral datum: a moral realm without wrongdoing may not be coherent. Thus, an adequate philosophic theory of morality ought to allow for it. But such a theory ought also to explain wrongdoing, both axiologically and causally. This is so if we take such a theory to have practical significance. Indeed, insofar as moral philosophy and its cognate areas have practical significance, explaining wrongdoing is arguably the most pressing practical issue for theory (...) construction in this domain. For a philosophical theory of morality to have practical significance, and, further, to have influence on behavior, it must be psychologically relevant to the organisms that it ranges over. That is, the concepts that the theory purports to explain must be plausibly realized within the psychology of the creatures for which the theory is intended. Any theory satisfying this constraint would then be in a position to illuminate those psychological features that are explanatory of blameworthy behavior. The argument presented here is that moral realism and its underlying moral psychology, cognitivism, face serious difficulties allowing for and explaining, both metaphysically and psychologically, such behavior. Moral cognitivism and realism fail to adequately account for this most fundamental of moral phenomena. Hence, a comprehensive understanding of moral experience is impossible within the cognitivist/realist perspective. (shrink)

This paper extends a stochastic theory for buffer fill distribution for multiple “on‘ and “off‘ sources to a mobile environment. Queue fill distribution is described by a set of differential equations assuming sources alternate asynchronously between exponentially distributed periods in “on‘ and “off‘ states. This paper includes the probabilities that mobile sources have links to a given queue. The sources represent mobile user nodes, and the queue represents the capacity of a switch. This paper presents a method of analysis which (...) uses mobile parameters such as speed, call rates per unit area, cell area, and call duration and determines queue fill distribution at the ATM cell level. The analytic results are compared with simulation results. (shrink)

In "Moral Arguments for Theistic Belief" Robert Merrihew Adams defends a theistic argument from the nature of morality according to which the existence of God is entailed by the divine-command theory, which Adams believes is our best account of morality. In reply I examine the four arguments for the modified divine-command theory that Adams develops in this and later papers, and I show that three of the arguments are much too weak to enable him to make a case for theism (...) in this way and that the fourth itself depends on the assumption that there is a God and so would render that case circular. (shrink)