Campaigning Culture and the Global Cold War: The Journals of the Congress for Cultural Freedom, edited by Giles Scott-Smith and Charlotte Lerg

A book review of: Giles Scott-Smith, and Charlotte Lerg, editors, Campaigning Culture and the Global Cold War: The Journals of the Congress for Cultural Freedom. Palgrave Macmillan, 2017

Mutations in DYNC2LI1 disrupt cilia function and cause short rib polydactyly syndrome.

The short rib polydactyly syndromes (SRPSs) are a heterogeneous group of autosomal recessive, perinatal lethal skeletal disorders characterized primarily by short, horizontal ribs, short limbs and polydactyly. Mutations in several genes affecting intraflagellar transport (IFT) cause SRPS but they do not account for all cases. Here we identify an additional SRPS gene and further unravel the functional basis for IFT. We perform whole-exome sequencing and identify mutations in a new disease-producing gene, cytoplasmic dynein-2 light intermediate chain 1, DYNC2LI1, segregating with disease in three families. Using primary fibroblasts, we show that DYNC2LI1 is essential for dynein-2 complex stability and that mutations in DYNC2LI1 result in variable length, including hyperelongated, cilia, Hedgehog pathway impairment and ciliary IFT accumulations. The findings in this study expand our understanding of SRPS locus heterogeneity and demonstrate the importance of DYNC2LI1 in dynein-2 complex stability, cilium function, Hedgehog regulation and skeletogenesis

Acylation of the Lipooligosaccharide of Haemophilus influenzae and Colonization: an htrB Mutation Diminishes the Colonization of Human Airway Epithelial Cells

Haemophilus influenzae is a commensal and opportunistic pathogen of the human airways. A number of surface molecules contribute to colonization of the airways by H. influenzae, such as adhesins, including structures found in the lipooligosaccharide (LOS). A human bronchiolar xenograft model was employed to investigate the host-bacterial interactions involved in the colonization of the airway by H. influenzae. Differential display was used to identify H. influenzae mRNA that reflect genes which were preferentially expressed in the xenograft compared to growth. Eleven mRNA fragments had consistent increased expression when the bacteria grew in xenografts. On sequencing these fragments, eight open reading frames were identified. Three of these had no match in the NCBI or the TIGR database, while an additional three were homologous to genes involved in heme or iron acquisition and utilization: two of the mRNAs encoded proteins homologous to enzymes involved in LOS biosynthesis: a heptosyl transferase (rfaF) involved in the synthesis of the LOS core and a ketodeoxyoctonate phosphate-dependent acyltransferase (htrB) that performs one of the late acylation reactions in lipid A synthesis. Inoculation of human bronchiolar xenografts revealed a significant reduction in colonization capacity by htrB mutants. In vitro, htrB mutants elicited lesser degrees of cytoskeletal rearrangement and less stimulation of host cell signaling with 16HBE14o- cells and decreased intracellular survival. These results implicate acylation of H. influenzae lipid A as playing a key role in the organisms' colonization of the normal airway

Regulating mobility in the Peruvian Andes: road safety, social hierarchies and governmentality in Cusco's rural provinces

Significant developments in road safety regulation have taken place in Peru during recent years, reflecting international efforts to reduce worldwide fatalities and injuries. A series of measures has sought to bring about transformations in governmentality among passengers on public transport. Seen ethnographically, these have had uneven success on the ground. In rural provinces of Cusco, situated histories and sociologies of mobility have sometimes led to ambivalence, unobtrusive resistance or reinforcement of discriminatory attitudes. This article explores how reception of the regulations has been refracted through class, ethnic and geographical divisions within Peruvian society, and argues for both the applied and theoretical utility of anthropological study of road safety governance

Panel. Cold War Faulkner

The Unlikely Patriot: Faulkner as Cold Warrior and Goodwill Ambassador for the U. S. Department of State / Deborah Cohn, Indiana UniversityBetween 1954 and 1961, Faulkner was recruited by the State Department to serve as good will ambassador and travel to “strategic” countries in Latin America, Asia, and Europe. These were the years of the Cold War, and as a Nobel Prize winner, Faulkner’s very person attested to the height of U.S. cultural achievement, while his style was figured as expressing artistic—and, by extension, democratic—freedom, as well as opposition to the social realism underpinning officially sanctioned Soviet politics and art. In this paper, I examine how constructions of Faulkner as both southerner and American interacted with one another on these tours. I further study how both his official travels and his pronouncements on race relations in the United States played directly into the State Department’s battle against communism. We—He and Us—Should Confederate: Intruder in the Dust, the Dixiecrat Campaign, and Faulkner\u27s Cold War Agenda / Alan Nadel, University of KentuckyIntruder in the Dust conducts a dialogue with Strom Thurman’s 1948 Dixiecrat Presidential Campaign, in the context of Cold War premises that inform Faulkner’s Nobel Prize speech. These premises, consistent with the policy of “containment,” made segregation a palpable threat to the nation’s ability to win the Cold War. To reimagine a nation in which the South participates in, rather than obstructs, the struggle to prevail over Communism, Faulkner stages, in the events surrounding the death of Vinson Gowrie, the end of the “separate but equal” doctrine supported the Supreme Court under Chief Justice Fred Vinson. In so doing, Faulkner adapts Great Expectations by inverting the power dynamics of Dickens’s novel, placing the child in the role of Magwitch who must devote his life to repaying the man—here Beauchamp—who saved him in the marshes. This inversion enables Faulkner to embrace the principles of the Dixiecrat campaign in order to renounce its objective. William Faulkner and the Problem of Cold War Modernism / Harilaos Harry Stecopoulos, University of IowaThis paper examines Faulkner’s most difficult experience with U.S. cultural diplomacy: his participation in the People-to-People (PTP) program. I first examine the absurd, yet pointed, letter with which Faulkner began his official duties for the PTP program, and then turn to his wry commentary on cold war modernism in The Mansion (1959). Through a linked reading of those two texts, I argue that Faulkner’s work for the PTP program prompted an attempt to reclaim from the cold war state the very modernist aesthetic he was meant to wield on behalf of the anti-Communist struggle. That attempt took fragmented shape, but in its very messiness, Faulkner’s riposte to the state made manifest the writer’s refusal to surrender his aesthetic to those cold warriors who found in modernism little more than a propagandistic symbol of artistic and popular freedom

Big Sur Visitor Characteristics and Wildland Fire Recreational Constraints

A study conducted with visitors to the Big Sur region of California during summer 2002 is presented. An onsite survey was administered to visitors to the U.S. Forest Service and California State Parks day-use and overnight facilities. Recreational constraints owing to wildland fire and fire management are detailed along with the effects of activity type, visitor demographics and other characteristics, and views of these constraints. Differences primarily exist in views of constraints related to regulations

Cartilage-selective genes identified in genome-scale analysis of non-cartilage and cartilage gene expression

<p>Abstract</p> <p>Background</p> <p>Cartilage plays a fundamental role in the development of the human skeleton. Early in embryogenesis, mesenchymal cells condense and differentiate into chondrocytes to shape the early skeleton. Subsequently, the cartilage anlagen differentiate to form the growth plates, which are responsible for linear bone growth, and the articular chondrocytes, which facilitate joint function. However, despite the multiplicity of roles of cartilage during human fetal life, surprisingly little is known about its transcriptome. To address this, a whole genome microarray expression profile was generated using RNA isolated from 18–22 week human distal femur fetal cartilage and compared with a database of control normal human tissues aggregated at UCLA, termed Celsius.</p> <p>Results</p> <p>161 cartilage-selective genes were identified, defined as genes significantly expressed in cartilage with low expression and little variation across a panel of 34 non-cartilage tissues. Among these 161 genes were cartilage-specific genes such as cartilage collagen genes and 25 genes which have been associated with skeletal phenotypes in humans and/or mice. Many of the other cartilage-selective genes do not have established roles in cartilage or are novel, unannotated genes. Quantitative RT-PCR confirmed the unique pattern of gene expression observed by microarray analysis.</p> <p>Conclusion</p> <p>Defining the gene expression pattern for cartilage has identified new genes that may contribute to human skeletogenesis as well as provided further candidate genes for skeletal dysplasias. The data suggest that fetal cartilage is a complex and transcriptionally active tissue and demonstrate that the set of genes selectively expressed in the tissue has been greatly underestimated.</p

Factors associated with recurrence and survival length following relapse in patients with neuroblastoma

Background: Despite therapeutic advances, survival following relapse for neuroblastoma patients remains poor. We investigated clinical and biological factors associated with length of progression-free and overall survival following relapse in UK neuroblastoma patients. Methods: All cases of relapsed neuroblastoma, diagnosed during 1990-2010, were identified from four Paediatric Oncology principal treatment centres. Kaplan-Meier and Cox regression analyses were used to calculate post-relapse overall survival (PROS), post-relapse progression-free survival (PRPFS) between relapse and further progression, and to investigate influencing factors. Results: One hundred eighty-nine cases were identified from case notes, 159 (84.0%) high risk and 17 (9.0%), unresectable, MYCN non-amplified (non-MNA) intermediate risk (IR). For high-risk patients diagnosed >2000, median PROS was 8.4 months (interquartile range (IQR)=3.0-17.4) and median PRPFS was 4.7 months (IQR=2.1-7.1). For IR, unresectable non-MNA patients, median PROS was 11.8 months (IQR 9.0-51.6) and 5-year PROS was 24% (95% CI 7-45%). MYCN amplified (MNA) disease and bone marrow metastases at diagnosis were independently associated with worse PROS for high-risk cases. Eighty percent of high-risk relapses occurred within 2 years of diagnosis compared with 50% of unresectable non-MNA IR disease. Conclusions: Patients with relapsed HR neuroblastomas should be treatment stratified according to MYCN status and PRPFS should be the primary endpoint in early phase clinical trials. The failure to salvage the majority of IR neuroblastoma is concerning, supporting investigation of intensification of upfront treatment regimens in this group to determine whether their use would diminish likelihood of relapse