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This article considers the construction and reimaging of the Banlieue space in Olivier Babinet’s 2016 film, Swagger. It is informed by Homi Bhabha’s concept of Third Space, and explores the ways in which the film challenges reductive representations of the Banlieue and its inhabitants, the violent rhetoric of French Universalism, and the myth of national identity. Swagger employs reflexive strategies that challenge dominant notions about the Banlieue space and population that are rooted in colonialism and White supremacy. In doing so, it reveals the connections between cinematic representation, systemic violence, and spatial transformation.

Background: Adolescents and young adults (AYA) with chronic conditions should acquire self-management skills as part of their healthcare transition (HCT) from pediatric to adult-focused care. HCT/self-management skills have the potential to help mitigate health disparities among minority AYA with chronic conditions. This study investigated school nurses’ practices promoting HCT/self-management skills in urban public schools. Methods: Seventy-nine nurses from three urban school districts in Massachusetts completed a survey of 32 Likert-type questions on HCT/self-management skills, eight demographic questions, and five open-ended practice questions assessing how often they have asked students with chronic conditions about HCT/self-management skills, based on the UNC TRxANSITION IndexTM. Results: Among the 79 school nurses who participated (response rate 76%), 67% never or rarely assessed students’ knowledge of HCT/self-management, and 90% would use a tool that promotes/measures HCT/self-management skills. Conclusion: In our study sample, most school nurses acknowledged the importance of assessing HCT/self-management skills. The majority favored using a tool to promote these skills.

Congenital hypothyroidism (CH) is a disorder of thyroid hormone deficiency which develops secondary to incomplete thyroid development or inadequate thyroid hormone production. State-mandated newborn screening throughout the United States has increased the detection rate of CH, allowing for early intervention. Although the overall mortality rate of CH is low, delayed or omitted treatment can lead to devastating neurocognitive outcomes. As such, CH is regarded as the leading cause of preventable intellectual disability in children. Early identification, facilitated by astute neonatal nursing and medical care, is contingent upon an active working knowledge of the disease process and awareness of the limitations of the newborn screen.

Sandhoff disease, one of the GM2 gangliosidoses, is a lysosomal storage disorder characterized by the absence of β-hexosaminidase A and B activity and the concomitant lysosomal accumulation of its substrate, GM2 ganglioside. It features catastrophic neurodegeneration and death in early childhood. How the lysosomal accumulation of ganglioside might affect the early development of the nervous system is not understood. Recently, cerebral organoids derived from induced pluripotent stem (iPS) cells have illuminated early developmental events altered by disease processes. To develop an early neurodevelopmental model of Sandhoff disease, we first generated iPS cells from the fibroblasts of an infantile Sandhoff disease patient, then corrected one of the mutant HEXB alleles in those iPS cells using CRISPR/Cas9 genome-editing technology, thereby creating isogenic controls. Next, we used the parental Sandhoff disease iPS cells and isogenic HEXB-corrected iPS cell clones to generate cerebral organoids that modeled the first trimester of neurodevelopment. The Sandhoff disease organoids, but not the HEXB-corrected organoids, accumulated GM2 ganglioside and exhibited increased size and cellular proliferation compared with the HEXB-corrected organoids. Whole-transcriptome analysis demonstrated that development was impaired in the Sandhoff disease organoids, suggesting that alterations in neuronal differentiation may occur during early development in the GM2 gangliosidoses.

The Learning Outcomes Assessment (LOA) movement seems rather innocuous. Teachers and administrators at colleges and universities are asked to articulate the goals, objectives, measures, and outcomes of the educational process at every level: from the classroom to the department to the institution as a whole. Educators engage in this process with the help of curriculum mapping or educational matrices or a host of other tools and templates provided by any number of readily available frameworks (see the website of the National Institute for Learning Outcomes Assessment for many examples). The information gathered is then used to evaluate curricula, programs, instructors, and institutions for purposes of internal review and external evaluation.

Intellectual disability (ID) is a heterogeneous condition arising from a variety of environmental and genetic factors. Among these causes are defects in transcriptional regulators. Herein, we report on two brothers in a nonconsanguineous family with novel compound heterozygous, disease-segregating mutations (NM_015979.3: [3656A > G];[4006C > T], NP_057063.2: [H1219R];[R1336X]) in MED23. This gene encodes a subunit of the Mediator complex that modulates the expression of RNA polymerase II-dependent genes. These brothers, who had profound ID, spasticity, congenital heart disease, brain abnormalities, and atypical electroencephalography, represent the first case of MED23-associated ID in a non-consanguineous family. They also expand upon the clinical features previously reported for mutations in this gene. © 2015 Wiley Periodicals, Inc.

This essay reviews Picturing the Modern Amazon, an exhibit of the representations of hypermuscular women presented at the New Museum of Contemporary Art in New York City from March 30 to June 25, 2000. Taking a sociohistorical approach, the essay situates the museum exhibit in the context of the development of American bodybuilding. The images of the Modern Amazons are linked to the intensification of capitalism and the subsequent growth of the bodybuilding, health, and fitness industries. From this perspective, we see that the Modern Amazons face a cultural paradox, for they are both gender-bending figures of muscular excess and a mainstream form of mass production; thus, they simultaneously overturn and recuperate gender norms. This essay explores the ways in which the exhibit itself embodies contradictions. In its attempt to celebrate the subversive aspects of hyper muscular females, the show decontexualizes the Modern Amazons, removing them from their muscle-building spaces and processes and rendering...

With recent advances in anabolic steroids, it is easy to see the gender- bending aspects of a professional bodybuilder’s body. The shaven and pumped-up pecs of male bodybuilders can grow droopy with gyne- comastia, a condition in which the body produces unwanted oestro- gen, inflating the pectoral area into protuberant lumps. Besides his developing “bitch tits,” as they are called in gym vernacular, a male bodybuilder’s testicles might shrink — in the words of one former Mr Universe,1 “to the size of cocktail peanuts” — even as his body elsewhere swells with giant balls of muscle. Similarly, a female bodybuilder, who in using steroids actually ingests male hormones, might lose the hair on her head, only to find it growing in patches on her face. Beyond the obvious development of her “manly” muscular body and the noticeable deepening of her voice, her menstruation might stop and her clitoris can elongate to the size of a small penis.

Two errors were made in this article. First, a reference was omitted from the second sentence of the fourth paragraph in the Introduction section. The sentence should read: SidD lacks any obvious sequence homology with the AT-N or other known proteins, although fold recognition analysis of the N-terminal portion of SidD predicted limited resemblance with members of the metal-dependent protein phosphatase (PPM) family (Rigden 2011). The reference is: Rigden DJ. (2011) Identification and modelling of a PPM protein phosphatase fold in the Legionella pneumophila deAMPylase SidD. FEBS Lett. 585:2749-54. doi: 10.1016/j.febslet.2011.08.006. Second, there were some omissions from the acknowledgements section, which should instead read as follows: We thank K. B. Decker, Y. Lin, and A.H. Gaspar for comments on the manuscript, and A. Vidaurrazaga for assistance with biochemical experiments. This study made use of the European Synchrotron Radiation Facility (ESRF, Grenoble, France) under the Block Allocation Group (BAG) MX1420, the Diamond Light Source (Oxfordshire, UK) under the rapid access mode MX7512 and BAG MX8302, the beamline PROXIMA1 at the SOLEIL synchrotron (Saint-Aubin, France), the X-ray crystallography platform and proteomics platform (member of CIBERehd and ProteoRed-ISCIII) at CIC bioGUNE (Derio, Spain), and the SGIker analytical facility at UPV/EHU (Leioa, Spain). We thank all the staff from these facilities for technical and human support. Access to synchrotron facilities was part funded by the the BioStruct-X project (proposal number 2460).

Inborn errors of metabolism (IEMs) that manifest primarily as psychiatric and behavioural symptoms in childhood are often mistaken for idiopathic primary psychiatric disorders. The pathophysiological basis of these symptoms may be overlooked until later in the disease course when neurological deficits become dominant; this results in a significant delay in establishing a proper diagnosis. To illustrate this, we describe two siblings who presented with behavioural issues and mild learning disabilities in childhood, and were consequently given multiple psychiatric diagnoses. In early adulthood, however, they manifested a rapid cognitive decline. Subsequent cranial MRI imaging revealed progressive brain iron accumulation in deep brain nuclei. Whole exome sequencing and biochemical investigation confirmed the diagnosis of mucopolysaccharidosis type IIIB. Their long diagnostic odyssey illustrates the importance of considering IEMs when assessing individuals with behavioural abnormalities and cognitive impairment.

The differentiation of the bacterium Bacillus subtilis into a dormant spore is among the most well-characterized developmental pathways in biology. Classical genetic screens performed over the past half century identified scores of factors involved in every step of this morphological process. More recently, transcriptional profiling uncovered additional sporulation-induced genes required for successful spore development. Here, we used transposon-sequencing (Tn-seq) to assess whether there were any sporulation genes left to be discovered. Our screen identified 133 out of the 148 genes with known sporulation defects. Surprisingly, we discovered 24 additional genes that had not been previously implicated in spore formation. To investigate their functions, we used fluorescence microscopy to survey early, middle, and late stages of differentiation of null mutants from the B. subtilis ordered knockout collection. This analysis identified mutants that are delayed in the initiation of sporulation, defective in membrane remodeling, and impaired in spore maturation. Several mutants had novel sporulation phenotypes. We performed in-depth characterization of two new factors that participate in cell–cell signaling pathways during sporulation. One (SpoIIT) functions in the activation of σE in the mother cell; the other (SpoIIIL) is required for σG activity in the forespore. Our analysis also revealed that as many as 36 sporulation-induced genes with no previously reported mutant phenotypes are required for timely spore maturation. Finally, we discovered a large set of transposon insertions that trigger premature initiation of sporulation. Our results highlight the power of Tn-seq for the discovery of new genes and novel pathways in sporulation and, combined with the recently completed null mutant collection, open the door for similar screens in other, less well-characterized processes., Transposon sequencing enables the recovery of virtually all previously characterized genes required for the differentiation of the bacterium Bacillus subtilis into a dormant spore and identifies 24 new ones., Author Summary When starved of nutrients, the bacterium Bacillus subtilis differentiates into a dormant spore that is impervious to environmental insults. Decades of research have uncovered over 100 genes required for spore formation. Molecular dissection of these genes has revealed factors that act at every stage of this developmental process. In this study, we used a high-throughput genetic screening method called transposon sequencing to assess whether there were any sporulation genes left to be discovered. This approach identified virtually all of the known sporulation genes, as well as 24 new ones. Furthermore, transposon sequencing enabled the discovery of two new sets of mutants in which the sporulation process was either delayed or accelerated. Using fluorescence microscopy, we determined the developmental stage at which each mutant was impaired and discovered mutants that are delayed in initiation of sporulation, or defective in morphogenesis, cell–cell signaling, or spore maturation. Our findings exemplify the utility of transposon sequencing to uncover new biology in well-studied processes, suggesting that it could similarly be used to identify novel genes required for other aspects of bacterial physiology, such as natural competence, stationary phase survival, or the responses to cell envelope stress and DNA damage.

Lysosomal storage diseases (LSD) are a heterogeneous group of approximately 60 disorders caused by lysosomal dysfunction. The significant variability in the age of onset, rate of progression, and symptomatology of these disorders can make diagnosis difficult, particularly for those LSD with later onset and delayed progression. We report a 25-year-old female who exhibited profound behavioral issues andmild learning disabilities in childhood. As a result, shewas diagnosed with multiple psychiatric disorders, and treated to attain acceptable behavior. In her mid-twenties, she manifested a rapid cognitive decline. Brain imaging studies showed moderate cortical atrophy and significant deposition of metal in several regions. Her family history was notable for an older sister with a very similar phenotype. In the absence of signs pathognomonic for their disease, we pursued whole exome sequencing of the family. This identified compound heterozygous mutations in NAGLU,which encodesN-acetyl-D-glucosaminidase. Biochemical studies confirmed enzyme deficiency diagnostic of mucopolysaccharidosis IIIB (Sanfilippo syndrome). Both the initial behavioral presentation and late onset of cognitive decline contributed to the 22-year diagnostic odyssey of this patient. This case demonstrates the importance of considering late-onset metabolic disorders, such as Sanfilippo syndrome, when evaluating individuals in a mental-health setting who exhibit behavioral abnormalities and learning disabilities.