Notes sobre la neotectònica al Vallès Oriental

Hi són descrits tot un seguit d'aspectes del modelat detallat del Valles Oriental associats a moviments del substrat. Aquests moviments del substrat són relacionables amb el funcionament recent de les falles pertanyents als dos sistemes de fractures, aproximadament ortogonals, presents al socol de la Depressió del Valles

Sobre la Geomorfologia del Vallès Oriental

Aquest treball és I'exposició dels principals resultats obtinguts a través de la cartografia geomorfològica, a escala 1:25.000, d'un sector del Vallès Oriental (nord-est de la Península Ibérica). S'hi adjunta, a més, un esquema geomorfològic.Hom hi descriu les formes, les formacions superficials i les alteracions correlatives a I'evolució del relleu del Vallès durant el Quaternari, que s'iriterpreten a la llum de les recents hipòtesis de I'evolució geomorfològica quaterniària de la Mediterrània occidental.En el domini de la dinàmica actual es ressenyen els efectes sobre el modelat dels diferents tipus de processos actuants, amb un petit comentari drls llits fluvials.Finalment s'hi fa una sèrie d'observacioris que fan sospitar l'actuació de la neotectònica recent a l'àrea estudiada

Fragmentation of magnetism in artificial kagome dipolar spin ice

Geometrical frustration in magnetic materials often gives rise to exotic, low-temperature states of matter, like the ones observed in spin ices. Here we report the imaging of the magnetic states of a thermally-active artificial magnetic ice that reveal the fingerprints of a spin fragmentation process. This fragmentation corresponds to a splitting of the magnetic degree of freedom into two channels and is evidenced in both real and reciprocal space. Furthermore, the internal organization of both channels is interpreted within the framework of a hybrid spin-charge model that directly emerges from the parent spin model of the kagome dipolar spin ice. Our experimental and theoretical results provide insights into the physics of frustrated magnets and deepen our understanding of emergent fields through the use of tailor-made magnetism.Comment: 9 pages, 5 figures. Published version available on the Nat. Comm. web site: http://www.nature.com/ncomms/2016/160513/ncomms11446/full/ncomms11446.htm

Sanfilippo syndrome: molecular basis, disease models and therapeutic approaches

Sanfilippo syndrome or mucopolysaccharidosis III is a lysosomal storage disorder caused by mutations in genes responsible for the degradation of heparan sulfate, a glycosaminoglycan located in the extracellular membrane. Undegraded heparan sulfate molecules accumulate within lysosomes leading to cellular dysfunction and pathology in several organs, with severe central nervous system degeneration as the main phenotypical feature. The exact molecular and cellular mechanisms by which impaired degradation and storage lead to cellular dysfunction and neuronal degeneration are still not fully understood. Here, we compile the knowledge on this issue and review all available animal and cellular models that can be used to contribute to increase our understanding of Sanfilippo syndrome disease mechanisms. Moreover, we provide an update in advances regarding the different and most successful therapeutic approaches that are currently under study to treat Sanfilippo syndrome patients and discuss the potential of new tools such as induced pluripotent stem cells to be used for disease modeling and therapy development

G protein-coupled receptors are dynamic regulators of digestion and targets for digestive diseases

G protein-coupled receptors (GPCRs) are the largest family of transmembrane signaling proteins. Within the gastrointestinal tract, GPCRs expressed by epithelial cells sense contents of the lumen, and GPCRs expressed by epithelial cells, myocytes, neurons, and immune cells participate in communication amongst cells. GPCRs control digestion, mediate digestive diseases, and coordinate repair and growth. GPCRs are the target of over one third of therapeutic drugs, including many drugs used to treat digestive diseases. Recent advances in structural, chemical, and cell biology research have revealed that GPCRs are not static binary switches that operate from the plasma membrane to control a defined set of intracellular signals. Rather, GPCRs are dynamic signaling proteins that adopt distinct conformations and subcellular distributions when associated with different ligands and intracellular effectors. An understanding of the dynamic nature of GPCRs has provided insights into the mechanism of activation and signaling of GPCRs, and has revealed opportunities for drug discovery. We review the allosteric modulation, biased agonism, oligomerization, and compartmentalized signaling of GPCRs that control digestion and digestive diseases. We highlight the implications of these concepts for the development of selective and effective drugs to treat diseases of the gastrointestinal tract

EXTL2 and EXTL3 inhibition with siRNAs as a promising substrate reduction therapy for Sanfilippo C syndrome

Sanfilippo syndrome is a rare lysosomal storage disorder caused by an impaired degradation of heparan sulfate (HS). It presents severe and progressive neurodegeneration and currently there is no effective treatment. Substrate reduction therapy (SRT) may be a useful option for neurological disorders of this kind, and several approaches have been tested to date. Here we use different siRNAs targeting EXTL2 and EXTL3 genes, which are important for HS synthesis, as SRT in Sanfilippo C patients' fibroblasts in order to decrease glycosaminoglycan (GAG) storage inside the lysosomes. The results show a high inhibition of the EXTL gene mRNAs (around 90%), a decrease in GAG synthesis after three days (30-60%) and a decrease in GAG storage after 14 days (up to 24%). Moreover, immunocytochemistry analyses showed a clear reversion of the phenotype after treatment. The in vitro inhibition of HS synthesis genes using siRNAs shown here is a first step in the development of a future therapeutic option for Sanfilippo C syndrome

Splicing therapeutics for patients affected by lysosomal storage disorders

In this study, we have used a modified U1 snRNA that completely matches the splice donor site of HGSNAT gene exon 2, which corrected the effect of the common 5’ splice site mutation c.234+1G>A in Mucopolysaccharidosis IIIC. In another approach using an antisense oligonucleotide (AO) we have succeeded in the correction of the c.66G>A splicing mutation in CSTB gene (Unverricht–Lundborg disease). Besides that, we have performed the functional analysis of some IDS gene splicing mutations (Mucopolysaccharidosis II) and used AOs to exploit an alternative therapy for one of those mutations (c.1122C>T on exon 8).Liliana Matos FCT grant (SFRH/BD/64592/2009)N/

Creating Airlines, videojoc de gestió de companyies aèries

BibliografiaEl projecte és la realització del disseny visual i lògic d'un videojoc enfocat a la gestió de companyies aèries, tant per a l'àmbit universitari com professional. El projecte desglossa les opcions del videojoc i els càlculs necessaris per a realitzar la seva funció.El proyecto es la realización del diseño visual y lógico de un videojuego enfocado a la gestión de compañías aéreas, tanto para el ámbito universitario como el profesional. El proyecto desglosa las opciones del videojuego y los cálculos necesarios para su función.The project is the accomplishment of the visual design and logical design of a video game focused on the management of airlines, both for the university area as for the professional one. The project explains the options of the video game and the necessary calculations for his function