Negotiating conservation: the construction of meaningful spaces in a world heritage debate

According to one’s personal biography, social background and the resultant degree of affectedness, a person has certain ideas about the meaning of, in our example, a World Heritage Site (WHS), what he or she can expect from it and what his or her relation to it can and should be. The handling of potentially different meaningful spaces is decisive, when it comes to the negotiation of pathways towards the sustainable development of a WHS region. Due to the fact that – in a pluralistic world – multiple realities exist, they have to be taken seriously and adequately addressed. In this article we identified the ways the Jungfrau-Aletsch- WHS was constructed by exploring the visual and verbal representations of the WHS during the decision-making process (1998-2001). The results demonstrate that in the visual representations (images), the WHS was to a large extent presented as an unspoiled natural environment similar to a touristy promotion brochure. Such a ‘picture-book’-like portrait has no direct link to the population’s daily needs, their questions and anxieties about the consequences of a WHS label. By contrast, the verbal representations (articles, letters-to-the-editor, comments) were dominated by issues concerning the economic development of the region, fears of disappropriation, and different views on nature. Whereas visual and verbal representations to a large extent differ significantly, their combination might have contributed to the final decision of the majority of people concerned to support the application for inscription of the Jungfrau-Aletsch-Bietschhorn region into the World Heritage list. The prominence of economic arguments and narratives about intergenerational responsibility in the verbal representations and their combination with the aesthetic appeal of the natural environment in the visual representations might have built a common meaningful space for one part of the population.

Men's Modesty, Religion, and the State: Spaces of Collision

This article examines religious practices in the United States, which govern modesty and other dress norms for men. I focus both on the spaces within which they most collide with regulatory regimes of the state and the legal implications of these norms, particularly for observant Muslim men. Undergirding the research are those ‘‘gender equality’’ claims made by many religious adherents, that men are required to maintain proper modesty norms just as are women. Also undergirding the research is the extensive anti-Islam bias in American culture today. The spaces within which men’s religiously proscribed dress and grooming norms are most at issue—indicated by First Amendment legal challenges to rights of religious practice—are primarily those state-controlled, total institutions Goffman describes, such as in the military and prisons. The implications of gendered modesty norms are important, as state control over religious expression in prisons, for example, is much more difficult to contest than in other spaces, although this depends entirely on who is doing the contesting and within which religious context. In American society today—and particularly within the context of growing Islamaphobia following the 9/11 attacks—the implications are greatest for those men practicing ‘‘prison Islam.’’

Characterization of novel StAR (steroidogenic acute regulatory protein) mutations causing non-classic lipoid adrenal hyperplasia

Context Steroidogenic acute regulatory protein (StAR) is crucial for transport of cholesterol to mitochondria where biosynthesis of steroids is initiated. Loss of StAR function causes lipoid congenital adrenal hyperplasia (LCAH). Objective StAR gene mutations causing partial loss of function manifest atypical and may be mistaken as familial glucocorticoid deficiency. Only a few mutations have been reported. Design To report clinical, biochemical, genetic, protein structure and functional data on two novel StAR mutations, and to compare them with published literature. Setting Collaboration between the University Children's Hospital Bern, Switzerland, and the CIBERER, Hospital Vall d'Hebron, Autonomous University, Barcelona, Spain. Patients Two subjects of a non-consanguineous Caucasian family were studied. The 46,XX phenotypic normal female was diagnosed with adrenal insufficiency at the age of 10 months, had normal pubertal development and still has no signs of hypergonodatropic hypogonadism at 32 years of age. Her 46,XY brother was born with normal male external genitalia and was diagnosed with adrenal insufficiency at 14 months. Puberty was normal and no signs of hypergonadotropic hypogonadism are present at 29 years of age. Results StAR gene analysis revealed two novel compound heterozygote mutations T44HfsX3 and G221S. T44HfsX3 is a loss-of-function StAR mutation. G221S retains partial activity (~30%) and is therefore responsible for a milder, non-classic phenotype. G221S is located in the cholesterol binding pocket and seems to alter binding/release of cholesterol. Conclusions StAR mutations located in the cholesterol binding pocket (V187M, R188C, R192C, G221D/S) seem to cause non-classic lipoid CAH. Accuracy of genotype-phenotype prediction by in vitro testing may vary with the assays employed.