Modelos celulares de inmunodeficiencias congénitas de linfocitos T: aplicación a estudios de expresión y señalización

Los linfocitos T desempeñan uno de los papeles más cruciales en la respuesta inmunológica: la respuesta adaptativa. La activación del linfocito T a través de su receptor, el TCR, desencadena una extensa red de señalización con decenas de proteínas intermediarias diferentes para generar respuestas efectoras adecuadas. El TCR es un complejo octamérico constituido por diferentes cadenas entre las que se encuentra CD247. La ausencia de cualquiera de los componentes del TCR se traduce en un defecto de desarrollo o función de los linfocitos T causante de SCID. Además, en los casos de deficiencias de CD247 se han descrito mutaciones somáticas minoritarias que son capaces de restaurar el defecto de expresión del TCR.

Modelos celulares de inmunodeficiencias congénitas de linfocitos T: aplicación a estudios de expresión y señalización

Tesis inédita de la Universidad Complutense de Madrid, Facultad de Medicina, Departamento de Inmunología, Oftalmología y ORL, leída el 26-11-2019Los linfocitos T desempeñan uno de los papeles más cruciales en la respuesta inmunológica: la respuesta adaptativa. La activación del linfocito T a través de su receptor, el TCR, desencadena una extensa red de señalización con decenas de proteínas intermediarias diferentes para generar respuestas efectoras adecuadas. El TCR es un complejo octamérico constituido por diferentes cadenas entre las que se encuentra CD247. La ausencia de cualquiera de los componentes del TCR se traduce en un defecto de desarrollo o función de los linfocitos T causante de SCID. Además, en los casos de deficiencias de CD247 se han descrito mutaciones somáticas minoritarias que son capaces de restaurar el defecto de expresión del TCR..T-lymphocytes play one of the most crucial roles in the immune response:the adaptive response. The activation of the T lymphocyte through its receptor, the TCR, triggers an extensive signalling network with dozens of different intermediate proteins to generate adequate effector responses. TCR is an octameric complex made up of different chains, including CD247. The absence of any of the components of the TCR results in a defect in the development or function of the Tlymphocytes causing SCID. In addition, in cases of CD247 deficiencies, minority somatic mutations have been described as capable of restoring the defect...Depto. de Inmunología, Oftalmología y ORLFac. de MedicinaTRUEunpu

Skewed TCR alpha, but not beta, gene rearrangements and lymphoma associated with a pathogenic TRAC variant

We report a non-consanguineous family from North-west India in which 3 siblings succumbed to a rare variant of combined immunodeficiency. All three had similar clinical and immunological profiles. However, the youngest child also developed Non-Hodgkin lymphoma in infancy. Genetic analysis revealed a previously reported variant in the T cell receptor alpha constant (TRAC) gene.Depto. de Inmunología, Oftalmología y ORLFac. de MedicinaTRUEpu

Human congenital T-cell receptor disorders

Immunodeficiencies of most T-cell receptor (TCR) components (TCRID) have been reported in almost 40 patients worldwide who have also, at times, shown signs of autoimmunity. We updated their clinical, immunological, and molecular features with an emphasis on practical diagnosis, as the range of the disorder grows in complexity with new partial defects. Cellular and animal models are also reviewed and in some cases reveal their limitations for predicting TCRID immunopathology

iTRAQ-Based Proteomic Profile Analysis of the Hepatopancreas of Caribbean Spiny Lobsters Infected WithPanulirus argusVirus 1 : Metabolic and Physiological Implications

The Caribbean spiny lobsterPanulirus argus(Latreille, 1084) sustains economically valuable fisheries throughout the wider Caribbean region. This species is currently affected by the pathogenic virusPanulirus argusVirus 1 (PaV1) that causes a systemic and chronic-degenerative infection in juvenile spiny lobstersP. argus. To date, there is no available information regarding the host alterations induced by this pathogen at the molecular level. In the present study, comparative proteomic analyses of the changes in the hepatopancreas between infected and non-infected juvenile lobsters were analyzed by isobaric tags for relative and absolute quantitation (iTRAQ) coupled to synchronous precursor selection (SPS)-based MS3. We identified a total of 636 proteins, being 68 down-regulated and 71 up-regulated proteins. Among the down-regulated proteins, we identified several enzymes involved in the metabolism of hormones and lipids, digestive proteases and glycosidases, while proteins associated with the histone core, protein synthesis, immune response and RNA regulation were up-regulated. Several misregulated enzymes involved in the regulation of neuromodulators were also identified. RT-qPCR assays were used to validate the expression of transcripts encoding for selected differential proteins that were in concordance to proteomic data, as well as the tendency observed in the enzymatic activities of trypsin, chymotrypsin, and glycosidase. In a similar way, we observed glycogen reduction in muscle, and an increase in plasma acylglycerides and glucose, which may be explained by proteomic data. This study provides the first insight into the molecular changes in the hepatopancreas of Caribbean spiny lobsters associated to PaV1 infection. Data provided herein would help to clarify the origin of the molecular misregulations observed at macroscopic level in this host-pathogen interaction

Poster competition as a learning tool in biomedical sciences

Se implementará una actividad de desarrollo en grupos de alumnos de pósters científicos y divulgativos. Dicho aprendizaje facilitará la adquisición de competencias personales e interpersonales por parte del alumnado y fomentar futuras interacciones internacionales.Depto. de Inmunología, Oftalmología y ORLDepto. de Química FísicaSección Deptal. de Biología Celular (Medicina)Fac. de MedicinaFac. de Ciencias QuímicasFALSEsubmitte

Primary T-cell immunodeficiency with functional revertant somatic mosaicism in CD247

Depto. de Inmunología, Oftalmología y ORLFac. de MedicinaTRUEMinisterio de Economía y Competitividad (MINECO)Instituto de Salud Carlos III (ISCIII)Comunidad de MadridFundacion LairUniversidad Complutense de Madridpu

The Sonozotz project: Assembling an echolocation call library for bats in a megadiverse country

Bat acoustic libraries are important tools that assemble echolocation calls to allow the comparison and discrimination to confirm species identifications. The Sonozotz project represents the first nation-wide library of bat echolocation calls for a megadiverse country. It was assembled following a standardized recording protocol that aimed to cover different recording habitats, recording techniques, and call variation inherent to individuals. The Sonozotz project included 69 species of echolocating bats, a high species richness that represents 50% of bat species found in the country. We include recommendations on how the database can be used and how the sampling methods can be potentially replicated in countries with similar environmental and geographic conditions. To our knowledge, this represents the most exhaustive effort to date to document and compile the diversity of bat echolocation calls for a megadiverse country. This database will be useful to address a range of ecological questions including the effects of anthropogenic activities on bat communities through the analysis of bat sound.</p