Molecular Characterization of Three Canine Models of Human Rare Bone Diseases: Caffey, van den Ende-Gupta, and Raine Syndromes.

One to two percent of all children are born with a developmental disorder requiring pediatric hospital admissions. For many such syndromes, the molecular pathogenesis remains poorly characterized. Parallel developmental disorders in other species could provide complementary models for human rare diseases by uncovering new candidate genes, improving the understanding of the molecular mechanisms and opening possibilities for therapeutic trials. We performed various experiments, e.g. combined genome-wide association and next generation sequencing, to investigate the clinico-pathological features and genetic causes of three developmental syndromes in dogs, including craniomandibular osteopathy (CMO), a previously undescribed skeletal syndrome, and dental hypomineralization, for which we identified pathogenic variants in the canine SLC37A2 (truncating splicing enhancer variant), SCARF2 (truncating 2-bp deletion) and FAM20C (missense variant) genes, respectively. CMO is a clinical equivalent to an infantile cortical hyperostosis (Caffey disease), for which SLC37A2 is a new candidate gene. SLC37A2 is a poorly characterized member of a glucose-phosphate transporter family without previous disease associations. It is expressed in many tissues, including cells of the macrophage lineage, e.g. osteoclasts, and suggests a disease mechanism, in which an impaired glucose homeostasis in osteoclasts compromises their function in the developing bone, leading to hyperostosis. Mutations in SCARF2 and FAM20C have been associated with the human van den Ende-Gupta and Raine syndromes that include numerous features similar to the affected dogs. Given the growing interest in the molecular characterization and treatment of human rare diseases, our study presents three novel physiologically relevant models for further research and therapy approaches, while providing the molecular identity for the canine conditions.

Pensar la Movida : Significaciones de subalternidad y resistencia en la cultura de la bailanta y la cumbia villera

Fil: Tranchini, Elina. Universidad Nacional de La Plata. Facultad de Humanidades y Ciencias de la Educación; Argentina.

Iruya entre dos miradas : Desde el documental etnográfico de Jorge Prelorán a la mirada subjetiva de Ulises de la Orden

En el presente trabajo se pretende comparar y distinguir, por medio análisis de audiovisuales diferentes y épocas de producción también distintas, como dos obras documentales plantearon su mirada y visión acerca de la localidad de Iruya. Con la representación de dos realizadores por un lado, Jorge Prelorán con su film etnográfico Iruya del año 1968 y por otro lado, Ulises de la Orden en la subjetividad del documental: Rio Arriba, Una Historia de terrazas, ingenios y volcanes, encontraron en Iruya, un paisaje transcultural como estrategia de resistencia más allá del audiovisual, que incorpora lo local y lo global, que busca solidaridades trasnacionales a través de su hibridismo, fruto de la ruptura de las fronteras culturales y la exaltan una vez más como una tierra de contrastes. Buscando con la comparación y contextualización del cine documental, entenderlos a ambos films como portavoces de la memoria a la vez que el discurso cinematográfico se convierte en narrador de la identidad e historia cultural de la comunidad de Iruya

Pensar la Movida : Significaciones de subalternidad y resistencia en la cultura de la bailanta y la cumbia villera

Fil: Tranchini, Elina. Universidad Nacional de La Plata. Facultad de Humanidades y Ciencias de la Educación; Argentina.

Las explotaciones familiares algodoneras en el Chaco argentino frente al avance de las innovaciones biotecnológicas : Reflexiones preliminares de un estudio de las relaciones entre género, tecnología y pobreza

Fil: Turkenich, Magalí. Universidad Nacional de La Plata. Facultad de Humanidades y Ciencias de la Educación; Argentina.

Internationalizing sales research:current status, opportunities and challenges

With economic activity in emerging markets growing at 40 percent, and with 10 percent and more of the firms in the Global Fortune 500 now headquartered in emerging economies, intense interest lies in the globalization of business activities, including the sales function. This systematic review of the international sales literature in a selection of the most influential journals explains, consolidates, and analyzes current knowledge. This paper also explores the challenges inherent in conducting international sales research, including conceptualization, research management, and data collection issues. Finally, we suggest ways to move forward for researchers in this field, including pertinent topics and how methodological and practical constraints might be addressed.

Logical Models of Composite Dynamic Objects Control

The questions of designing multicriteria control systems on the basis of logic models of composite dynamic objects are considered.