Pituitary blastoma: a pathognomonic feature of germ-line DICER1 mutations.

Individuals harboring germ-line DICER1 mutations are predisposed to a rare cancer syndrome, the DICER1 Syndrome or pleuropulmonary blastoma-familial tumor and dysplasia syndrome [online Mendelian inheritance in man (OMIM) #601200]. In addition, specific somatic mutations in the DICER1 RNase III catalytic domain have been identified in several DICER1-associated tumor types. Pituitary blastoma (PitB) was identified as a distinct entity in 2008, and is a very rare, potentially lethal early childhood tumor of the pituitary gland. Since the discovery by our team of an inherited mutation in DICER1 in a child with PitB in 2011, we have identified 12 additional PitB cases. We aimed to determine the contribution of germ-line and somatic DICER1 mutations to PitB. We hypothesized that PitB is a pathognomonic feature of a germ-line DICER1 mutation and that each PitB will harbor a second somatic mutation in DICER1. Lymphocyte or saliva DNA samples ascertained from ten infants with PitB were screened and nine were found to harbor a heterozygous germ-line DICER1 mutation. We identified additional DICER1 mutations in nine of ten tested PitB tumor samples, eight of which were confirmed to be somatic in origin. Seven of these mutations occurred within the RNase IIIb catalytic domain, a domain essential to the generation of 5p miRNAs from the 5' arm of miRNA-precursors. Germ-line DICER1 mutations are a major contributor to PitB. Second somatic DICER1 "hits" occurring within the RNase IIIb domain also appear to be critical in PitB pathogenesis.

PPAR beta/delta Agonists Modulate Platelet Function via a Mechanism Involving PPAR Receptors and Specific Association/Repression of PKC alpha-Brief Report

Objectives-Peroxisome proliferator-activated receptor beta/delta (PPAR beta/delta) is a nuclear receptor found in platelets. PPAR beta/delta agonists acutely inhibit platelet function within a few minutes of addition. As platelets are anucleated, the effects of PPAR beta/delta agonists on platelets must be nongenomic. Currently, the particular role of PPAR beta/delta receptors and their intracellular signaling pathways in platelets are not known. Methods and Results-We have used mice lacking PPAR beta/delta (PPAR beta/delta(-/-)) to show the effects of the PPAR beta/delta agonist GW501516 on platelet adhesion and cAMP levels are mediated specifically by PPAR beta/delta, however GW501516 had no PPAR beta/delta-specific effect on platelet aggregation. Studies in human platelets showed that PKC alpha, which can mediate platelet activation, was bound and repressed by PPAR beta/delta after platelets were treated with GW501516. Conclusions-These data provide evidence of a novel mechanism by which PPAR receptors influence platelet activity and thereby thrombotic risk. (Arterioscler Thromb Vasc Biol. 2009; 29: 1871-1873.)

Abolishing mammography screening programs?

Biller-Andorno and Jüni (2014), in a widely debated commentary published in the May 22 issue of the New England Journal of Medicine, accept the concept that mammography every 2 years from age 50 can decrease breast cancer mortality by 20%, that is, from five to four deaths per 1000 women over a 10-year period. Both the absolute and the relative risk of breast cancer death may vary depending on the baseline mortality rates in various populations and on the impact of screening mammography in reducing breast cancer mortality, which may well vary around the 20% estimate adopted. We accept, therefore, that there are still uncertainties in the absolute and relative impact of mammography screening on breast cancer mortality, given the different study schemes and mammography intervals, the differences in populations, and the continuous improvements in technology (Warner, 2011; Independent UK Panel on Breast Cancer Screening, 2012). We also agree on the observation that mammography has an appreciable impact on breast cancer mortality (Bosetti et al., 2012), but clearly a much smaller one on total mortality.

Mixed and Hybrid Models of Public Service Delivery

This symposium presents research from different contexts to improve our collective understanding of a variety of aspects of mixed forms of service delivery, be they mixed contracting at the level of the market (which is more common in the U.S.), or mixed management and ownership at the level of the firm (which is more common in Europe). The articles included in this special symposium examine the factors that give rise to mixed forms of service delivery (e.g., economic and fiscal stress, regulatory flexibility, geography, management) and how these factors impact their design and operation. Articles also explore the performance of mixed forms of service delivery relative to more conventional arrangements like contracted or direct service delivery. The articles contribute to a better theoretical and conceptual understanding of mixed/hybrid forms of services delivery.

Understanding change processes in business networks : a study of convergence in Finnish telecommunications 1985-2005

Aktörer inom telekommunikationsbranschen i Finland har genomgått en intensiv förändring under de senaste 25 åren, från 1980-talets självständiga företag till företag beroende av varandra, och även av aktörer inom närliggande branscher. I dag skapas telekommunikationsmarknaden inte endast av operatörerna, utan också av mediebolag (t.ex. MTV Media) och IT-företag (t.ex. TietoEnator). Gränserna mellan olika industrier håller därmed på att suddas ut - ett fenomen som allmänt benämns som teknologisk konvergens. Konvergens innebär att någonting integreras; det kan handla om t.ex. teknologier (telefoni och Internet), företag (AOL och Time Warner), industrier (telekom, media och IT-branscherna), tjänster (mobilt TV), produkter (PDA) osv. Detta innebär att ytterst få telekomaktörer ensamma kan vidareutveckla marknaden och tekniska lösningar. Samarbete mellan aktörer krävs; mobiltelefontillverkare, innehållsproducenter, operatörer osv. bör intesifiera sitt samarbete för att kunna erbjuda attraktiva tjänster och produkter till kunder och slutanvändare. Avhandlingen fokuserar speciellt på affärsnätverk och samarbetsmönster mellan nätverksaktörer som medel för att få tillgång till resurser som krävs i en konvergenskarakteriserad affärsomgivning. Avhandlingen lyfter fram vad den teknologiska konvergensen har inneburit för telekomaktörer, dvs. att företag tvingats förändra sina strategier och verksamhetsmodeller. För många företag i branschen har anpassningen till konvergenstänkande varit utmanande, och i vissa fall kan man till och med tala om att företagen upplevt en identitetskris. Den utförda forskningen visar att konvergens uppfattas på marknaden som en pågående förändringsprocess, där varje telekomaktör är tvungen att utvärdera sin roll och position i relation till andra aktörer inom branschen. Konvergensprocesser forsätter i framtiden med ökad intensitet. Aktörerna skapar medvetet sin omgivning genom att agera i olika roller, som kan sträcka sig över industrigränser. Avhandlingen påvisar även att externa händelser och industrikontexten påverkar dynamiken i ett affärsnätverk.

Conhecimentos em atenção pré-hospitalar e suporte básico de vida por estudantes recém-ingressos de medicina

Reconhecer sinais iminentes de morte em vítimas de trauma, acidente vascular encefálico e acidentes domésticos constitui parte do Suporte Básico de Vida (SBV), ação fundamental à prática médica. Objetivou-se investigar o conhecimento em manobras no SBV e urgências domiciliares entre estudantes recém-ingressos de Medicina. Estudo quantitativo, analítico e quase-experimental, realizado com estudantes do primeiro semestre 2012.2 do curso de Medicina de universidade pública do Ceará - Campus Cariri em Barbalha (CE), Brasil. Os dados foram coletados em fases. Na primeira etapa, aplicou-se um questionário com 40 perguntas de múltipla escolha sobre manobras de atendimento pré-hospitalar em SBV como pré-teste de forma presencial. A segunda etapa consistiu num minicurso de 20 horas com profissional treinado. Na terceira fase, utilizou-se pós-teste para comparar as questões acertadas antes e depois do curso. Quanto aos conhecimentos adquiridos, houve acréscimo nos temas traumatismo raquimedular, liberação de vias aéreas e cuidados domiciliares. Há necessidade de realizar cursos com maior carga horária para fundamentar uma prática médica centrada nos temas de menor rendimento dos estudantes.

Managing Change: An Organizational Outline for Reimagining the Digital Repository Infrastructure at The Ohio State University Libraries

Poster at Open Repositories 2014, Helsinki, Finland, June 9-13, 2014

The grab of the world's land and water resources

In this paper, I review recent developments in global political economy and political economy of development that have captured inter alia the attention of agrarian political economists. I do so through the periscope of two recent publications by Fred pearce, Great Britain's leading eco journalist and an edited volume by Tony Allann, Martin Keulertz, Suvi Sojamo and Jeroen Warner, scholars trained in different disciplines and based at various universities in the UK, the netherlands, and Finland. The account of the pace, places, and perpetrators, procedures, and problems of this particular agrarian model provides fodder for the further development of a locus classicus on what is happening to the land question in this current moment under the capitalist order, a shorthand for which is 'water and land grab'.

Dianna Hostetter funeral invitation, 1861

The invitation is a request of Jacob Hostetter to his friends and acquaintances to attend the funeral of his wife, Dianna on Tuesday, October 29, 1861. Dianna, also known as Dinah, was the second daughter of Joseph and Mary Heslop Van Every. She was born in 1831 and married Jacob Hostetter of Grantham Township. Jacob died a year later, leaving two children, Joseph Blain Hostetter (1860-1896) and Laura Diana Hostetter (1861-1933). Jacob was the son of Capt. Herman Hostetter of Ten Mile Creek who had died from wounds received at the Battle of Queenston Heights. Jacob and Dianna Hostetter are buried in the Warner Cemetery, Niagara Falls, Ont.

William Van Every fonds, 1784, 1804-1806, 1815-1816, 1828, 1832, n.d.

William Van Every, son of McGregory and Mary Wilcox (Jaycocks) Van Every, was born in New York state in 1765. During the Revolutionary War he joined Butler’s Rangers and served under Captain John McDonnell. He was granted three lots of land in the Township of Niagara, with additional lands granted at later dates. William married Elizabeth, daughter of George Young. Elizabeth was the widow of Col. Frederick Dochstader and mother of Catherine Dochstader, b. 1781. William Van Every died in 1832, his wife Elizabeth in 1851. Both are buried in the Warner Cemetery, in present day Niagara Falls. The children of William Van Every and Elizabeth Young were Mary, Elizabeth, Phoebe, John, Peter, William, Rebecca, Samuel and Joseph. Source: Mary Blackadar Piersol, The Records of the Van Every Family, Toronto : Best Printing, 1947. And, Patricia M. Orr, Historic Woodend, sponsored by Niagara Peninsula Conservation Authority, 1980?

Schram Family of Wainfleet Township documents, 1839-1901 (not inclusive)

George William Schram (1809-1885), son of Garrett Schram and Leah Van Etten, married Orpha Pearson on Nov.13, 1835. His son Marsena John Schram (farmer) was born in May of 1840, in Canada. He died on Nov. 17, 1926 in Wexford County, Michigan. He was married in 1867 to Sarah (1825-1887).Marsena married again on April 18, 1910 to Ann Clarinda Warner (1861-1924). He was working as a carpenter at this time. They had another son, William who was born about 1838 and he married Sabina Chambers on Jan. 21, 1862. The 1861 census for Wainfleet lists siblings of Marsena John Schram as Sarah J. (age 14), Georgiana (age 5), and William (age 21). The Schrams lived on Concession 5 and owned approximately 144 acres of land. David Thompson was born Feb. 4, 1873 and died Feb. 19, 1951. He married Sally Ann Wilson on Sept. 7, 1825 in Pelham. She died about 1840 in Indiana Ontario (near Cayuga). Lemuel Victor Hogue was born Dec.1, 1854 and died Jan. 12, 1929. He was married to Elizabeth Wills who was born Aug. 2, 1861 and died Mar. 8, 1926. Sources: http://www.findagrave.com/cgi-bin/fg.cgi?page=gr&GRid=99294842 http://wc.rootsweb.ancestry.com/cgi-bin/igm.cgi?op=GET&db=seadragon5&id=I91708

Rôle de l’extrémité C-terminale dans l’expression du canal calcique Cav1.2 à la membrane plasmique

Le canal calcique de type L, Cav1.2, joue un rôle clé dans le couplage excitation-contraction des myocytes ventriculaires. Il a été montré que la sous-unité Cavα1 était sujette à l’épissage alternatif et que ce phénomène pouvait mener à une protéine tronquée en C-terminal au niveau de l’exon 45 (Liao, Yong et al. 2005). D’autres groupes ont étudié différentes délétions au niveau de l’extrémité C-terminale (De Jongh, Warner et al. 1991; Gao, Cuadra et al. 2001). Les courants mesurés dans la configuration cellule entière, était significativement plus grands que le canal « pleine longueur ». Nous avons décidé de tester certaines de ces délétions (ΔC2030, ΔC1935, ΔC1856, ΔC1733, ΔC1700) en présence ou en absence de la sous-unité auxiliaire Cavβ3, susceptible d’interagir avec l’extrémité C-terminale de la sous-unité Cavα1 par l’intermédiaire de son domaine SH3 (Lao, Kobrinsky et al. 2008). Les résultats obtenus dans les ovocytes de Xénope ont mis en évidence que les sous-unités Cavα1.2 tronquées montraient des courants globaux plus élevés que le canal « pleine longueur » en présence de la sous-unité auxiliaire Cavβ3 et que les sous-unités Cavα1.2 tronquées donnaient des courants en absence de la sous-unité Cavβ3 contrairement à la sous-unité Cavα1.2 « pleine longueur ». Afin de vérifier si l’augmentation des courants macroscopiques était le résultat d’une augmentation du nombre de sous-unités Cavα1.2 à la membrane, nous avons choisi de quantifier la fluorescence spécifiquement due à cette sous-unité en utilisant la méthode de cytométrie de flux (FACS : « Fluorescence Activated Cell Sorting »). L’épitope HA a été inséré dans une région extracellulaire de la sous-unité Cavα1 du canal calcique Cav1.2 et un anticorps anti-HA couplé au FITC (« Fluorescein IsoThioCyanate ») a été utilisé pour observer la fluorescence. Nos résultats confirment que la sous-unité Cavα1-HA du canal calcique Cav1.2, s’exprime à la membrane plasmique en présence de la sous-unité auxiliaire Cavβ3, et qu’en absence de celle-ci, ne s’exprime que peu ou pas à la membrane. Les mêmes résultats ont été obtenus pour les trois délétions testées dans les mêmes conditions soit Cavα1.2-HA ΔC1935, Cavα1.2-HA ΔC1856 et Cavα1.2-HA ΔC1733. Ensemble, ces résultats suggèrent que l’augmentation des courants macroscopiques observés après une délétion partielle du C-terminal n’est pas causée par une augmentation du nombre de protéines Cavα1.2 à la membrane.

Le studio d’enregistrement comme lieu d’expérimentation, outil créatif et vecteur d’internationalisation : Stonetree Records et la paranda garifuna en Amérique centrale

La version intégrale de cette thèse est disponible uniquement pour consultation individuelle à la Bibliothèque de musique de l’Université de Montréal (www.bib.umontreal.ca/MU).

La juventud a través del cine (1953-1959).

Resumen tomado de la publicación