Search results for "CEOS"
showing 10 items of 20 documents
SANS (USH1G) regulates pre-mRNA splicing by mediating the intra-nuclear transfer of tri-snRNP complexes
2021
Abstract Splicing is catalyzed by the spliceosome, a compositionally dynamic complex assembled stepwise on pre-mRNA. We reveal links between splicing machinery components and the intrinsically disordered ciliopathy protein SANS. Pathogenic mutations in SANS/USH1G lead to Usher syndrome—the most common cause of deaf-blindness. Previously, SANS was shown to function only in the cytosol and primary cilia. Here, we have uncovered molecular links between SANS and pre-mRNA splicing catalyzed by the spliceosome in the nucleus. We show that SANS is found in Cajal bodies and nuclear speckles, where it interacts with components of spliceosomal sub-complexes such as SF3B1 and the large splicing cofact…
NineTeen Complex-subunit Salsa is required for efficient splicing of a subset of introns and dorsal-ventral patterning
2020
© 2020 Rathore et al. This article is distributed exclusively by the RNASociety for the first 12 months after the full-issue publication date (see http://rnajournal.cshlp.org/site/misc/terms.xhtml). After 12 months, it is available under a Creative Commons License (Attribution-NonCommercial 4.0 International), as described at http://creativecommons.org/licenses/by-nc/4.0/.
Investigating the Molecular Mechanism of H3B-8800: A Splicing Modulator Inducing Preferential Lethality in Spliceosome-Mutant Cancers.
2021
The SF3B1 protein, part of the SF3b complex, recognizes the intron branch point sequence of precursor messenger RNA (pre-mRNA), thus contributing to splicing fidelity. SF3B1 is frequently mutated in cancer and is the target of distinct families of splicing modulators (SMs). Among these, H3B-8800 is of particular interest, as it induces preferential lethality in cancer cells bearing the frequent and highly pathogenic K700E SF3B1 mutation. Despite the potential of H3B-8800 to treat myeloid leukemia and other cancer types hallmarked by SF3B1 mutations, the molecular mechanism underlying its preferential lethality towards spliceosome-mutant cancer cells remains elusive. Here, microsecond-long a…
HNRNPR Variants that Impair Homeobox Gene Expression Drive Developmental Disorders in Humans
2019
The heterogeneous nuclear ribonucleoprotein (HNRNP) genes code for a set of RNA-binding proteins that function primarily in the spliceosome C complex. Pathogenic variants in these genes can drive neurodegeneration, through a mechanism involving excessive stress-granule formation, or developmental defects, through mechanisms that are not known. Here, we report four unrelated individuals who have truncating or missense variants in the same C-terminal region of hnRNPR and who have multisystem developmental defects including abnormalities of the brain and skeleton, dysmorphic facies, brachydactyly, seizures, and hypoplastic external genitalia. We further identified in the literature a fifth ind…
Deregulated Splicing Is a Major Mechanism of RNA-Induced Toxicity in Huntington's Disease.
2019
Huntington's disease (HD) is caused by an expanded CAG repeat in the huntingtin (HTT) gene, translating into an elongated polyglutamine stretch. In addition to the neurotoxic mutant HTT protein, the mutant CAG repeat RNA can exert toxic functions by trapping RNA-binding proteins. While few examples of proteins that aberrantly bind to mutant HTT RNA and execute abnormal function in conjunction with the CAG repeat RNA have been described, an unbiased approach to identify the interactome of mutant HTT RNA is missing. Here, we describe the analysis of proteins that preferentially bind mutant HTT RNA using a mass spectrometry approach. We show that (I) the majority of proteins captured by mutant…
Integration of CSR into CEOs’ compensation: effects on social and financial performance of French listed firms
2023
Based on the stakeholder model of corporate governance, this research analyzes the relationship between the integration of corporate social responsibility (CSR) into CEO compensation and corporate social and financial performance, in firms listed on France’s SBF 120 stock market between 2015 and 2019. The results of panel data regressions show that indexing CEO compensation on CSR criteria only influence corporate social performance Moreover, social performance has a positive impact on financial performance and a mediating effect between the integration of CSR criteria into CEO compensation and financial performance.
Parasitismo por cestodos en Artemia spp. y su implicación en la invasión biológica de Artemia franciscana en la región Mediterránea
2015
La presente Tesis Doctoral trata del fenómeno del parasitismo en el crustáceo Artemia y la implicación de los cestodos en el proceso de invasión biológica de la especie americana Artemia franciscana Kellogg, 1906 en la región Mediterránea. El crustáceo Artemia (Branchiopoda, Anostraca) es el invertebrado más conspicuo de los ecosistemas acuáticos hipersalinos de todo el mundo y un valioso recurso trófico para las poblaciones de aves acuáticas que residen en dichos ecosistemas. El uso generalizado de la especie americana A. franciscana en la acuicultura y acuariofilia han provocado su introducción y expansión fuera de su área nativa, localizándose en la actualidad en todos los continentes y …
Marcadores genéticos dopaminérgicos, serotoninérgicos y de la monoamino-oxidasa (MAO) relacionados con la adicción a los opiáceos, a la cocaína y al …
2013
Introducción: la genética está experimentando un avance espectacular y se postula como una herramienta importante para aportar nuevos descubrimientos en trastornos complejos como son las adicciones. En el presente trabajo se ha realizado un estudio de asociación genética de polimorfismos VNTR, un estudio familiar y un estudio mixto de asociación genético-familiar en una muestra de policonsumidores con adicción al alcohol, la cocaína y los opiáceos. Material y métodos: en una muestra de 302 policonsumidores, se recogieron y genotiparon muestras a partir de células epiteliales de la mejilla para observar las frecuencias de marcadores de las vías dopaminérgica, serotoninérgica y de la MAO en l…
Kauppaneuvos Soili Suonoja (1944–), Amica Ravintolat Oy:n toimitusjohtaja
2011
On Line Validation Exercise (OLIVE): A Web Based Service for the Validation of Medium Resolution Land Products. Application to FAPAR Products
2014
International audience; The OLIVE (On Line Interactive Validation Exercise) platform is dedicated to the validation of global biophysical products such as LAI (Leaf Area Index) and FAPAR (Fraction of Absorbed Photosynthetically Active Radiation). It was developed under the framework of the CEOS (Committee on Earth Observation Satellites) Land Product Validation (LPV) sub-group. OLIVE has three main objectives: (i) to provide a consistent and centralized information on the definition of the biophysical variables, as well as a description of the main available products and their performances (ii) to provide transparency and traceability by an online validation procedure compliant with the CEO…