Mutation Update of the CLCN5 Gene Responsible for Dent Disease 1

Dent disease is a rare X-linked tubulopathy characterized by low molecular weight proteinuria, hypercalciuria, nephrocalcinosis and/or nephrolithiasis, progressive renal failure, and variable manifestations of other proximal tubule dysfunctions. It often progresses over a few decades to chronic renal insufficiency, and therefore molecular characterization is important to allow appropriate genetic counseling. Two genetic subtypes have been described to date: Dent disease 1 is caused by mutations of the CLCN5 gene, coding for the chloride/proton exchanger ClC-5; and Dent disease 2 by mutations of the OCRL gene, coding for the inositol polyphosphate 5-phosphatase OCRL-1. Herein, we review previously reported mutations (n = 192) and their associated phenotype in 377 male patients with Dent disease 1 and describe phenotype and novel (n = 42) and recurrent mutations (n = 24) in a large cohort of 117 Dent disease 1 patients belonging to 90 families. The novel missense and in-frame mutations described were mapped onto a three-dimensional homology model of the ClC-5 protein. This analysis suggests that these mutations affect the dimerization process, helix stability, or transport. The phenotype of our cohort patients supports and extends the phenotype that has been reported in smaller studies.

siRNA mediated knockdown of tissue factor expression in pigs for xenotransplantation.

Acute vascular rejection (AVR), in particular microvascular thrombosis, is an important barrier to successful pig-to-primate xenotransplantation. Here, we report the generation of pigs with decreased tissue factor (TF) levels induced by small interfering (si)RNA-mediated gene silencing. Porcine fibroblasts were transfected with TF-targeting small hairpin (sh)RNA and used for somatic cell nuclear transfer. Offspring were analyzed for siRNA, TF mRNA and TF protein level. Functionality of TF downregulation was investigated by a whole blood clotting test and a flow chamber assay. TF siRNA was expressed in all twelve liveborn piglets. TF mRNA expression was reduced by 94.1 ± 4.7% in TF knockdown (TFkd) fibroblasts compared to wild-type (WT). TF protein expression in PAEC stimulated with 50 ng/mL TNF-α was significantly lower in TFkd pigs (mean fluorescence intensity TFkd: 7136 ± 136 vs. WT: 13 038 ± 1672). TF downregulation significantly increased clotting time (TFkd: 73.3 ± 8.8 min, WT: 45.8 ± 7.7 min, p < 0.0001) and significantly decreased thrombus formation compared to WT (mean thrombus coverage per viewing field in %; WT: 23.5 ± 13.0, TFkd: 2.6 ± 3.7, p < 0.0001). Our data show that a functional knockdown of TF is compatible with normal development and survival of pigs. TF knockdown could be a valuable component in the generation of multi-transgenic pigs for xenotransplantation.

Na 1 Nachlass Max Horkheimer, 657 - "The Philosophy of Western Society", "Bericht über die Aktivitäten des Instituts für Sozialforschung für Robert M. MacIver", "Über die Tätigkeit des Instituts für Sozialforschung", "Stipendiaten des Instituts für Sozialforschung: Forschungsberichte", "Research Projects of the International Institut of Social Research" (p. IX 56-IX 59.2)

Über das Forschungsprogramm: The Philosophy of Western Society. Teilstück des allgemeinen Forschungsprogramms, veröffentlicht unter dem Untertitel: "The Philosophy of Social Science", in: "International Institute of Social Research. A Report of Its History, Aims and Activites, 1933-1938", New York (1938?), S. 19, Typoskript mit eigenhändiger Korrektur, 2 Blatt; Bericht über die Aktivitäten des Instituts für Sozialforschung für Robert M. MacIver, 1938-39: 1. Bericht vom 7.12.1939; a) Typoskript, englisch, 9 Blatt; b) Typoskript, als Brief von Pollock an Robert M. MacIver, Typoskript 10 Blatt; c) Entwurf Typoskript, englisch, 15 Blatt; d) Entwurf Typoskript, deutsch, 17 Blatt; MacIver, Robert M.: 1 Brief mit Unterschrift an Friedrich Pollock, New York, 27.04.1938, 1 Blatt; "Some data on the Institut`s Staff and Activities", 11.03.1938, zwei Typoskripte, je 2 Blatt; Über die Tätigkeiten des Instituts für Sozialforschung. Verschiedene Berichte. 1939; Aufstellung der Forschungsgebiete verschiedener Mitarbeiter des Instituts. Ohne Datum, Typoskript, 9 Blatt; Aufstellung der Forschungsgebiete verschiedener Mitarbeiter des Institutes, aus einem Bericht. Typoskript, 7 Blatt; Bericht an den Präsidenten des Columbia University. Typoskript, 2 Blatt; "Contribution of Dr. Franz Neumann to the Round Table Discussion, Chicago, Social Science Reaserach Building" Dezember 1939; a) Typoskript mit Handschriftlichen Korrekturen, 3 Blatt; b) Typoskript, 3 Blatt; "Statment on the objectives of the International Institut of Social Research". Typoskript, 2 Blatt; Stipendiaten des Instituts für Sozialforschung: Forschungsberichte, Ende 1939; Adorno, Theodor W.: a) Typoskript, englisch, mit eigenhändigen Korrekturen, 4 Blatt; b) Typoskript, deutsch, 3 Blatt; Beck, Maximilian: "Geschichte des Begriffs der Vernunft von Platon bis Husserl (Outline)". Typoskript, englisch und deutsch, mit handschriftlichen Korrekturen, 7 Blatt; Flechtheim, Ossip K.: Typoskript mit handschriftlichen Ergänzungen, 1 Blatt; Fried, Hans Ernest: Typoskript, 1 Blatt, 16.11.1939; Grossmann, Henryk: "Capitalism in the 13th Century"; a) Typoskript, englisch, mit handschriftlichen Korrekturen, 6 Blatt; b) Typoskript, englisch, 6 Blatt; c) Typoskript, deutsch, 4 Blatt; Grossmann, Henryk: "The Classical Theory and Marxism"; a) Typoskript, englisch, mit handschriftlichen Korrekturen, 2 Blatt; b) Typoskript, deutsch, mit handschriftlichen Korrekturen, 2 Blatt; Kirchheimer, Otto: Manuskript, 1 Blatt; Lauterbach, Albert: a) Typoskript, englisch, mit handschriftlichen Korrekturen, 2 Blatt; b) Typoskript, deutsch, mit handschriftlichen Korrekturen, 1 Blatt; Marcuse, Herbert: Typoskript mit handschriftlichen Korrekturen, 2 Blatt; Neumann, Franz L.: Typoskript, 2 Blatt; Wittfogel, Karl August: Typsokript, 2 Blatt; Zilsel, Edgar: Typoskript, 4 Blatt; Research Projects of the International Institute of Social Research. nicht vor 1939; Beschreibung der Forschungsprojekte: Pollock, Friedrich: "Economic and Social Cosequences of a Prepardness Economy". Neumann, Franz L.: "The Rule of Law". Fromm, Erich: "The German Worker in the Weiman Republic". Kirchheimer,Otto: "Criminal Law and Social Structure". Marcuse, Herbert: " A Text and Source Book for the History of Philosophy". Fromm, Erich: "Character Structure of Modern Man". Neumann, Franz L.: "The Theory and Practice of European Labor Law". Wittfogel, Karl August; Wittfogel-Lang, Olga: "The Chinese Family"; Dasselbe wie in "Beschreibung der Forschungsprojekte", ausgenommen Pollock, Friedrich: "Economic and Social Cosquences of a Prepardness Economy" und Fromm, Erich: "The German Worker in the Weimar Republic", zusätzlich Kirchner, Otto; Weil, Felix: "Changes in Social Stratification, National Income, and Living Standards of Germany since 1933"; 1 Ordner, Typoskript mit eigenhändigen Korrekturen, 49 Blatt;

Family Strengths: A Long Tradition

Alvin Sallee, Angelo Giardino, and Robert Sanborn discuss the latest issue of Journal of Family Strengths, celebrating the centennial of the Children's Bureau.

Family Preservation Journal, Summer 1995, Volume 1. (Entire issue)

Entire issue (large pdf file) Articles include: Family Preservation and Support: Past, Present, and Future. Katharine Hooper-Briar,C. Anne Broussard, John Ronnau, and Alvin Sallee An Examination of Treatment Fidelity in an Intensive Family Preservation Program. Marianne Berry Intensive Family Reunification Services: A Conceptual Framework and Case Example. Elaine Walton, Mark W. Fraser, Catherin Harlin, and Robert E. Lewis Intensive Family Preservation Services: Do They Have Any Impact on Family Functioning? Roy W. Rodenhiser, Joseph Chandy, Kazi Ahmed Institutionalizing Intensive Family Preservation Services: A Strategy for Creating Staffing Standards Based on Projections of At-Risk Children from Referral sources. Robert E. Lewis

Understanding and Fostering Family Resilience

This paper examines a model of resilience and provides a fictional case example from the classical musical, Fiddler on the Roof along with a discussion of how this model may be helpful in assisting families at various levels of functioning to bounce back and perhaps even experience growth through facing difficult challenges.

Family Preservation Journal, 2003, Volume 7. (Entire issue)

Entire issue (large pdf file) Articles include: Social Workers' Perceptions of Family Preservation Programs. Elaine M Maccio, David Skiba, Howard J Doueck, Karen A. Randolph, Elisabeth A. Weston, and Lorie E. Anderson Targeting Special Populations for Family Preservation: The Influence of Worker Competence and Organizational Culture. Ramona W: Denby, Keith A. Alford, and Carla M Curtis Understanding and Fostering Family Resilience. Robert G. Blair Walking Our Talk in the Neighborhoods: Building Professional/Natural Helper Partnerships. Jill Kinney and Margaret Trent Intersystem Collaboration: A Statewide Initiative to Support Families. Elizabeth M Tracy, David E. Biegel, Ann C. Rebeck, and Jeffrey A. Johnsen

Properties of seawater and particulate matter (fluorescence) from a WETLabs Eco-FL sensor mounted on the continuous surface water sampling system during the Tara Oceans expedition 2009-2013

The Tara Oceans Expedition (2009-2013) sampled the world oceans on board a 36 m long schooner, collecting environmental data and organisms from viruses to planktonic metazoans for later analyses using modern sequencing and state-of-the-art imaging technologies. Tara Oceans Data are particularly suited to study the genetic, morphological and functional diversity of plankton. The present data set provides continuous measurements made with a WETLabs Eco-FL sensor mounted on the flowthrough system between June 4th, 2011 and March 30th, 2012. Data was recorded approximately every 10s. Two issues affected the data: 1. Periods when the water 0.2µm filtered water were used as blanks and 2. Periods where fluorescence was affected by non-photochemical quenching (NPQ, chlorophyll fluorescence is reduced when cells are exposed to light, e.g. Falkowski and Raven, 1997). Median data and their standard deviation were binned to 5min bins with period of light/dark indicated by an added variable (so that NPQ affected data could be neglected if the user so chooses). Data was first calibrated using HPLC data collected on the Tara (there were 36 data within 30min of each other). Fewer were available when there was no evident NPQ and the resulting scale factor was 0.0106 mg Chl m-3/count. To increase the calibration match-ups we used the AC-S data which provided a robust estimate of Chlorophyll (e.g. Boss et al., 2013). Scale factor computed over a much larger range of values than HPLC was 0.0088 mg Chl m-3/count (compared to 0.0079 mg Chl m-3/count based on manufacturer). In the archived data the fluorometer data is merged with the TSG, raw data is provided as well as manufacturer calibration constants, blank computed from filtered measurements and chlorophyll calibrated using the AC-S. For a full description of the processing of the Eco-FL please see Taillandier, 2015.

(Table S1) Age determination of ODP Site 202-1240

Interannual-decadal variability in the equatorial Pacific El Niño-Southern Oscillation (ENSO) induces climate changes at global scale, but its potential influence during past global climate change is not yet well constrained. New high-resolution eastern equatorial Pacific proxy records of thermocline conditions present new evidence of strong orbital control in ENSO-like variability over the last 275,000 years. Recurrent intervals of saltier thermocline waters are associated with the dominance of La Niña-like conditions during glacial terminations, coinciding with periods of low precession and high obliquity. The parallel dominance of d13C-depleted waters supports the advection of Antarctic origin waters toward the tropical thermocline. This "oceanic tunneling" is proposed to have reinforced orbitally induced changes in ENSO-like variability, composing a complex high- and low-latitude feedback during glacial terminations.

“O CRISTO AFOGADO: UMA NÃO-CRISTOLOGIA” RELIGIÃO, LITERATURA E PÓS-COLONIALISMO

O objetivo dessa tese é aprofundar, a partir do discurso pós-colonial, uma crise na perspectiva teológica da libertação. Esta promoveu, na década de 1970, uma reviravolta nos estudos teológicos no terceiro mundo. Para tanto, leremos um conto de Gabriel García Márquez chamado “El ahogado más hermosodel mundo” (1968) analizando e avaliando as estratégias políticas e culturais ali inscritas. Para levar a frente tal avaliação é preciso ampliar o escopo de uma visão que divide o mundo em secular/religioso, ou em ideias/práticas religiosas e não religiosas, para dar passo a uma visão unificada que compreende a mundanalidade, tanto do que é catalogado como ‘religioso’ quanto do que se pretende ‘não religioso’. A teologia/ciências da religião, como discurso científico sobre a economia das trocas que lidam com visões, compreensões e práticas de mundo marcadas pelo reconhecimento do mistério que lhes é inerente, possuem um papel fundamental na compreensão, explicitação, articulação e disponibilização de tais forças culturais. A percepção de existirem elementos no conto que se relacionam com os símbolos sobre Jesus/Cristo nos ofereceu um vetor de análise; entretanto, não nos deixamos limitar pelos grilhões disciplinares que essa simbologia implica. Ao mesmo tempo, esse vínculo, compreendido desde a relação imperial/colonial inerente aos discursos e imagens sobre Jesus-Cristo, embora sem centralizar a análise, não poderia ficar intocado. Partimos para a construção de uma estrutura teórica que explicitasse os valores, gestos, e horizontes mundanos do conto, cristológicos e não-cristológicos, contribuindo assim para uma desestabilização dos quadros tradicionais a partir dos quais se concebem a teologia e as ciências da religião, a obra de García Márquez como literatura, e a geografia imperial/colonial que postula o realismo ficcional de territórios como “América Latina”. Abrimos, assim, um espaço de significação que lê o conto como uma “não-cristologia”, deslocando o aprisionamento disciplinar e classificatório dos elementos envolvidos na análise. O discurso crítico de Edward Said, Homi Bhabha e GayatriSpivak soma-se à prática teórica de teólogas críticas feministas da Ásia, da África e da América Latina para formular o cenário político emancipatório que denominaremos teologia crítica secular.

GESTÃO DO CONHECIMENTO: UM ESTUDO SOBRE A RELAÇÃO ENTRE A LIDERANÇA TRANSFORMACIONAL, A CONVERSÃO DO CONHECIMENTO E A EFICÁCIA ORGANIZACIONAL

Este estudo teve como objetivo principal analisar a relação entre a Liderança Transformacional, a Conversão do Conhecimento e a Eficácia Organizacional. Foram considerados como pressupostos teóricos conceitos consolidados sobre os temas desta relação, além de recentes pesquisas já realizadas em outros países e contextos organizacionais. Com base nisto identificou-se potencial estudo de um modelo que relacionasse estes três conceitos. Para tal considera-se que as organizações que buscam atingir Vantagem Competitiva e incorporam a Knowledge-Based View possam conquistar diferenciação frente a seus concorrentes. Nesse contexto o conhecimento ganha maior destaque e papel protagonista nestas organizações. Dessa forma criar conhecimento através de seus colaboradores, passa a ser um dos desafios dessas organizações ao passo que sugere melhoria de seus indicadores Econômicos, Sociais, Sistêmicos e Políticos, o que se define por Eficácia Organizacional. Portanto os modos de conversão do conhecimento nas organizações, demonstram relevância, uma vez que se cria e se converte conhecimentos através da interação entre o conhecimento existente de seus colaboradores. Essa conversão do conhecimento ou modelo SECI possui quatro modos que são a Socialização, Externalização, Combinação e Internalização. Nessa perspectiva a liderança nas organizações apresenta-se como um elemento capaz de influenciar seus colaboradores, propiciando maior dinâmica ao modelo SECI de conversão do conhecimento. Se identifica então na liderança do tipo Transformacional, características que possam influenciar colaboradores e entende-se que esta relação entre a Liderança Transformacional e a Conversão do Conhecimento possa ter influência positiva nos indicadores da Eficácia Organizacional. Dessa forma esta pesquisa buscou analisar um modelo que explorasse essa relação entre a liderança do tipo Transformacional, a Conversão do Conhecimento (SECI) e a Eficácia Organizacional. Esta pesquisa teve o caráter quantitativo com coleta de dados através do método survey, obtendo um total de 230 respondentes válidos de diferentes organizações. O instrumento de coleta de dados foi composto por afirmativas relativas ao modelo de relação pesquisado com um total de 44 itens. O perfil de respondentes concentrou-se entre 30 e 39 anos de idade, com a predominância de organizações privadas e de departamentos de TI/Telecom, Docência e Recursos Humanos respectivamente. O tratamento dos dados foi através da Análise Fatorial Exploratória e Modelagem de Equações Estruturais via Partial Least Square Path Modeling (PLS-PM). Como resultado da análise desta pesquisa, as hipóteses puderam ser confirmadas, concluindo que a Liderança Transformacional apresenta influência positiva nos modos de Conversão do Conhecimento e que; a Conversão do Conhecimento influencia positivamente na Eficácia Organizacional. Ainda, concluiu-se que a percepção entre os respondentes não apresenta resultado diferente sobre o modelo desta pesquisa entre quem possui ou não função de liderança.