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Msh2 Acts in Medium-Spiny Striatal Neurons as an Enhancer of CAG Instability and Mutant Huntingtin Phenotypes in Huntington’s Disease Knock-In Mice
Marina Kovalenko, Ella Dragileva, Jason St. Claire, Tammy Gillis, Jolene R. Guide, Jaclyn New, Hualing Dong, Raju Kucherlapati, Melanie H. Kucherlapati, Michelle E. Ehrlich, Jong-Min Lee, Vanessa C. Wheeler
PLOS ONE , 2012, DOI: 10.1371/journal.pone.0044273
Abstract: The CAG trinucleotide repeat mutation in the Huntington’s disease gene (HTT) exhibits age-dependent tissue-specific expansion that correlates with disease onset in patients, implicating somatic expansion as a disease modifier and potential therapeutic target. Somatic HTT CAG expansion is critically dependent on proteins in the mismatch repair (MMR) pathway. To gain further insight into mechanisms of somatic expansion and the relationship of somatic expansion to the disease process in selectively vulnerable MSNs we have crossed HTT CAG knock-in mice (HdhQ111) with mice carrying a conditional (floxed) Msh2 allele and D9-Cre transgenic mice, in which Cre recombinase is expressed specifically in MSNs within the striatum. Deletion of Msh2 in MSNs eliminated Msh2 protein in those neurons. We demonstrate that MSN-specific deletion of Msh2 was sufficient to eliminate the vast majority of striatal HTT CAG expansions in HdhQ111 mice. Furthermore, MSN-specific deletion of Msh2 modified two mutant huntingtin phenotypes: the early nuclear localization of diffusely immunostaining mutant huntingtin was slowed; and the later development of intranuclear huntingtin inclusions was dramatically inhibited. Therefore, Msh2 acts within MSNs as a genetic enhancer both of somatic HTT CAG expansions and of HTT CAG-dependent phenotypes in mice. These data suggest that the selective vulnerability of MSNs may be at least in part contributed by the propensity for somatic expansion in these neurons, and imply that intervening in the expansion process is likely to have therapeutic benefit.
A novel approach to investigate tissue-specific trinucleotide repeat instability
Jong-Min Lee, Jie Zhang, Andrew I Su, John R Walker, Tim Wiltshire, Kihwa Kang, Ella Dragileva, Tammy Gillis, Edith T Lopez, Marie-Josee Boily, Michel Cyr, Isaac Kohane, James F Gusella, Marcy E MacDonald, Vanessa C Wheeler
BMC Systems Biology , 2010, DOI: 10.1186/1752-0509-4-29
Abstract: Here we describe a novel approach to gain insight into the factors responsible for the tissue specificity of somatic instability. Using accurate genetic knock-in mouse models of HD, we developed a reliable, high-throughput method to quantify tissue HD CAG repeat instability and integrated this with genome-wide bioinformatic approaches. Using tissue instability quantified in 16 tissues as a phenotype and tissue microarray gene expression as a predictor, we built a mathematical model and identified a gene expression signature that accurately predicted tissue instability. Using the predictive ability of this signature we found that somatic instability was not a consequence of pathogenesis. In support of this, genetic crosses with models of accelerated neuropathology failed to induce somatic instability. In addition, we searched for genes and pathways that correlated with tissue instability. We found that expression levels of DNA repair genes did not explain the tissue specificity of somatic instability. Instead, our data implicate other pathways, particularly cell cycle, metabolism and neurotransmitter pathways, acting in combination to generate tissue-specific patterns of instability.Our study clearly demonstrates that multiple tissue factors reflect the level of somatic instability in different tissues. In addition, our quantitative, genome-wide approach is readily applicable to high-throughput assays and opens the door to widespread applications with the potential to accelerate the discovery of drugs that alter tissue instability.Expansions of trinucleotide repeat sequences over certain thresholds cause more than 30 human diseases including Huntington's disease (HD), a number of spinocerebellar ataxias (SCAs), myotonic dystrophy 1 (DM1), and fragile X syndrome. Interestingly, expanded trinucleotide repeat sequences undergo progressive, expansion-biased tissue-specific somatic instability [1-6]. As the severity of these disorders is highly dependent on repeat length,
International Crises, Characterization of Contagion and Social Well-Being in Developing Countries: A Theoretical Model  [PDF]
Giscard Assoumou Ella
Theoretical Economics Letters (TEL) , 2014, DOI: 10.4236/tel.2014.41011
Abstract:

This paper has two objectives: to characterize the exposure of developing countries to the international income, prices and monetary shocks and to calculate the social well-being in the period of contagion. Firstly, we develop a theoretical model with a world composed of two countries (developed and developing countries) and measure the level of the exposure of income in developing country to the external shocks trough external trade, international tourism, migrant transfers, external debt, foreign aid, FDI and other private financial flow channels. And, we characterize imported inflation in studying the effect of the international shocks on real exchange rate. Secondly, we search the social well-being. The results suggest that economic disequilibrium in developing country is socially optimal and that the dependence of its income to the domestic industry is necessary to reduce contagion. This conclusion is important for African countries because they import finished products for the households’ consumption. In these conditions, they are exposed to the imported inflation. The domestic monetary and tax policies are not adapted to fight against inflation. Also, they produce and export raw materials essentially toward industrialized economies. Thus, they must develop the domestic industry in order to influence the demand (or prices) of raw materials and to substitute imports by domestic goods in period of hyper-inflation in advanced economies in order to reduce imported inflation.

Mismatch Repair Genes Mlh1 and Mlh3 Modify CAG Instability in Huntington's Disease Mice: Genome-Wide and Candidate Approaches
Ricardo Mouro Pinto,Ella Dragileva,Andrew Kirby,Alejandro Lloret,Edith Lopez,Jason St. Claire,Gagan B. Panigrahi,Caixia Hou,Kim Holloway,Tammy Gillis,Jolene R. Guide,Paula E. Cohen,Guo-Min Li,Christopher E. Pearson,Mark J. Daly,Vanessa C. Wheeler
PLOS Genetics , 2013, DOI: 10.1371/journal.pgen.1003930
Abstract: The Huntington's disease gene (HTT) CAG repeat mutation undergoes somatic expansion that correlates with pathogenesis. Modifiers of somatic expansion may therefore provide routes for therapies targeting the underlying mutation, an approach that is likely applicable to other trinucleotide repeat diseases. Huntington's disease HdhQ111 mice exhibit higher levels of somatic HTT CAG expansion on a C57BL/6 genetic background (B6.HdhQ111) than on a 129 background (129.HdhQ111). Linkage mapping in (B6x129).HdhQ111 F2 intercross animals identified a single quantitative trait locus underlying the strain-specific difference in expansion in the striatum, implicating mismatch repair (MMR) gene Mlh1 as the most likely candidate modifier. Crossing B6.HdhQ111 mice onto an Mlh1 null background demonstrated that Mlh1 is essential for somatic CAG expansions and that it is an enhancer of nuclear huntingtin accumulation in striatal neurons. HdhQ111 somatic expansion was also abolished in mice deficient in the Mlh3 gene, implicating MutLγ (MLH1–MLH3) complex as a key driver of somatic expansion. Strikingly, Mlh1 and Mlh3 genes encoding MMR effector proteins were as critical to somatic expansion as Msh2 and Msh3 genes encoding DNA mismatch recognition complex MutSβ (MSH2–MSH3). The Mlh1 locus is highly polymorphic between B6 and 129 strains. While we were unable to detect any difference in base-base mismatch or short slipped-repeat repair activity between B6 and 129 MLH1 variants, repair efficiency was MLH1 dose-dependent. MLH1 mRNA and protein levels were significantly decreased in 129 mice compared to B6 mice, consistent with a dose-sensitive MLH1-dependent DNA repair mechanism underlying the somatic expansion difference between these strains. Together, these data identify Mlh1 and Mlh3 as novel critical genetic modifiers of HTT CAG instability, point to Mlh1 genetic variation as the likely source of the instability difference in B6 and 129 strains and suggest that MLH1 protein levels play an important role in driving of the efficiency of somatic expansions.
Os sefarditas em Israel: o sionismo do ponto de vista das vítimas judaicas
Shohat, Ella;
Novos Estudos - CEBRAP , 2007, DOI: 10.1590/S0101-33002007000300006
Abstract: this article aims to contemplate an issue seldom mentioned in alternative critical discourse concerning israel and zionism: the presence of arab or oriental jews, the sephardi jews, coming largely from arab and moslem countries. a broader analysis must include negative consequences of zionism not only to palestinian people, but also to the sephardi jews. the zionist denial of the arab-moslem and palestinian east has as its corollary the denial of the mizrahim (the "eastern ones"), who, like the palestinians, have also been stripped of the right of self-representation.
Des-orientar Cleópatra: um tropo moderno da identidade
Shohat, Ella;
Cadernos Pagu , 2004, DOI: 10.1590/S0104-83332004000200002
Abstract: this article proposes a study about cleopatra's representation throughout the last century, situating the debate on her looks and origins among colonial domination, anti-colonial struggles and post-colonial racial frictions that, as it tries to demonstrate, add another dimension towards understanding the investment in cleopatra's identity.
ECONOMIC ETHICS: APPLIED AND PROFESSIONAL CHARACTER
Ella Gordova
Sovremennye Issledovania Social?nyh Problem , 2012,
Abstract: In given article economic ethics are considered as set of norms of behavior of the businessman, the requirements shown by a cultural society to its style of work, to character of dialogue between participants of business, to their social shape. The conclusion becomes that economic ethics have applied character in relation to theoretical, to obschenormativnoy ethics, hence, represent section of applied ethics. On the other hand, the specific standard maintenance characterizes economic ethics as ethics professional.
Die anderen Ausdrucksweisen: subtile Offensiven Different Modes of Expression: Subtle Offensives
Ella Jasiowka
querelles-net , 2002,
Abstract: Was passiert, wenn der Herrscher des filmischen Blickes“ eine Frau ist? Anhand zweier jeweils sehr unterschiedlicher Beispiele weiblicher“ Filmkunst zeigt die Autorin, dass es n tig ist, herk mmliche Interpretationsmuster zu überwinden, um dem Filmschaffen einzelner Künstler und Künstlerinnen auf den Grund zu gehen. Da es nicht die feministische Kunst“ an sich gibt, mu man für das Werk so unterschiedlicher Künstlerinnen wie Sally Potter und Jane Campion jeweils unterschiedliche Schlüssel suchen, um ihnen gerecht zu werden. Dies tut Radkiewicz in ihrem Werk – aus feministischer Perspektive. Sie holt jeweils verschieden weit aus, wenn es darum geht, künstlerische Herkunft, Biographie, Vorbilder und Ziele der Künstlerinnen zu deren Werk in Bezug zu setzten. Ohne zu bahnbrechend neuen Erkenntnissen zu kommen, gelingt es ihr doch, ein komplexes Bild der von ihr behandelten Filmemacherinnen und den Filmen zu entwerfen, das einen umfassenden Gesamteindruck vermittelt. Die Kürze des Buches bringt dabei eine Konzentration mit sich, die zu einer Weiterbesch ftigung und Vertiefung einl dt. What happens if the filmmaker directing the audience’s gaze is a woman? Radkiewicz uses two very different examples of “female” film art to portray the two directions into which this kind of undertaking can lead. In so doing, she demonstrates that traditional methods of analysis no longer suffice to thoroughly examine the work of an artist. Since there is no neatly defined category of “feminist art,” one has to take into consideration different kinds of approaches in order to do justice to the work of film directors like Jane Campion and Sally Potter. Radkiewicz does exactly that in her work, and thus provides an analysis that is informed by a feminist perspective. She includes the filmmakers’ artistic trajectory, background, biography, and goals into her interpretation of Campion’s and Potter’s films. While Radkiewicz does not re-invent or expand feminist theory, she succeeds at providing a complex portrayal of the two filmmakers and their work, and offering a good overview over their work. The conciseness of Radkiewicz’s book provides a useful introduction to these issues and invites its readers to further explorations.
A vinda para a América: reflex es sobre perda de cabelos e de memória
SHOHAT ELLA
Revista Estudos Feministas , 2002,
Abstract:
A Note on Conserved Charges of Asymptotically Flat and Anti-de Sitter Spaces in Arbitrary Dimensions
Ella Jamsin
Physics , 2007, DOI: 10.1007/s10714-008-0640-6
Abstract: The calculation of conserved charges of black holes is a rich problem, for which many methods are known. Until recently, there was some controversy on the proper definition of conserved charges in asymptotically anti-de Sitter (AdS) spaces in arbitrary dimensions. This paper provides a systematic and explicit Hamiltonian derivation of the energy and the angular momenta of both asymptotically flat and asymptotically AdS spacetimes in any dimension D bigger or equal to 4. This requires as a first step a precise determination of the asymptotic conditions of the metric and of its conjugate momentum. These conditions happen to be achieved in ellipsoidal coordinates adapted to the rotating solutions.The asymptotic symmetry algebra is found to be isomorphic either to the Poincare algebra or to the so(D-1, 2) algebra, as expected. In the asymptotically flat case, the boundary conditions involve a generalization of the parity conditions, introduced by Regge and Teitelboim, which are necessary to make the angular momenta finite. The charges are explicitly computed for Kerr and Kerr-AdS black holes for arbitrary D and they are shown to be in agreement with thermodynamical arguments.
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