196 research outputs found

    Recent advances in the exploration of the bile salt export pump (BSEP/ABCB11) function

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    ntroduction: The bile salt export pump (BSEP/ABCB11), residing in the apical membrane of hepatocyte, mediates the secretion of bile salts into the bile. A range of human diseases is associated with the malfunction of BSEP, including fatal hereditary liver disorders and mild cholestatic conditions. Manifestation of these diseases primarily depends on the mutation type; however, other factors such as hormonal changes and drug interactions can also trigger or influence the related diseases. Areas covered: Here, we summarize the recent knowledge on BSEP by covering its transport properties, cellular localization, regulation and major mutations/polymorphisms, as well as the hereditary and acquired diseases associated with BSEP dysfunction. We discuss the different model expression systems employed to understand the function of the BSEP variants, their drug interactions and the contemporary therapeutic interventions. Expert opinion: The limitations of the available model expression systems for BSEP result in controversial conclusions, and obstruct our deeper insight into BSEP deficiencies and BSEP-related drug interactions. The knowledge originating from different methodologies, such as clinical studies, molecular genetics, as well as in vitro and in silico modeling, should be integrated and harmonized. Increasing availability of robust molecular biological tools and our better understanding of the mechanism of BSEP deficiencies should make the personalized, mutation-based therapeutic interventions more attainable

    A KARSZTVIDÉKEK JELENTŐSÉGE AZ ERDÉLYI-SZIGETHEGYSÉG TURISZTIKAI POTENCIÁLJÁBAN

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    The Apuseni Mountains is a very interesting unit among the mountains of Romania and South-Eastern Europe. Due to the variety of the geological structure its relief is diversified. Although the karstic rocks occupy only 6% of the whole region, and another 9% are occupied by partially karstifying rocks, we consider that the karst has an important role in these mountains. Based on touristic maps we created a database with almost all natural touristic attractions. We ranked these sights according to their importance from locally significant points to internationally well-known tourist targets. More than 80% of the almost 600 sights are situated on karst. This shows the potential importance of karst in tourism. We present the most important touristic maps of the region, mostly those presenting karstic regions. We compare the tourist potential of karst with statistic data regarding the overnight stays spent by tourists in different accommodations

    Morfometrijska analiza ponikava i krških reljefnih oblika Biokova (Hrvatska) temeljena na digitalnoj analizi reljefa i terenskom istraživanju

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    Biokovo Mt is part of the Outer Dinarides, in a coastal position with a NW-SE main tectonic orientation. The highest peak called Sveti [Sv.] Jure (1762 m) towers above a karst plateau of 1300-1400 m mean elevation. Both of the long margins of Biokovo are characterized by steep and high relief energy rockwalls formed along tectonic fault lines. The plateau is intensely karstified with diverse karren forms, large dolines and several shaft caves. Its unique karstmorphological features are the relatively deep dolines with steep walls, so the landscape is characterized mainly by the sharp interdoline ridges and pyramidal peaks instead of negative doline forms. Based on the previous observation, the Biokovo plateau can be considered a „polygonal karst”. The geomorphological map of a study area from this plateau has been elaborated, based on field observations and topographical maps. After digitizing the contour lines, we have created the digital elevation model (DEM) of the study area and carried out sophisticated doline-morphometrical analysis using the DEM. The most important and unique feature of the landscape, the polygonal structure, has been derived from the DEM using GIS hydrologic modelling tools.Planina Biokovo nalazi se na obali Jadranskog mora i dio je Vanjskih Dinarida s tipičnim dinarskim smjerom pružanja SZ–JI. Najviši vrh Biokova Sv. Jure (1762 m) izdiže se iznad krške zaravni koja se nalazi na visini od 1300 do 1400 m. Sjeveroistočna i jugozapadna padina Biokova nastale su duž glavnih rasjeda, vrlo su strme i velike su reljefne energije. Krška zaravan građena je od jurskih i krednih vapnenaca i dolomita te je intenzivno okršena. To se očituje u bogatstvu krških reljefnih oblika: škrapa, vrlo velikih ponikava i jama. Posebnost su krškog reljefa Biokova duboke ponikve vrlo strmih padina, tako da je krajobraz istraživanog područja više obilježen oštrim i uskim grebenima i visokim piramidalnim vrhovima koji međusobno odjeljuju ponikve nego udubljenjima. Na temelju dosadašnjih istraživanja biokovsku kršku zaravan možemo smatrati "poligonalnim kršem". Na osnovi terenskoga kartiranja i različitih analiza topografse karte načinjena je geomorfološka karta istraživanog područja površine od 3 km2 koje se nalazi jugozapadno od vrha Sv. Jure. Na temelju digitaliziranih izohipsa izrađen je digitalni model reljefa (DEM) istraživanog područja, koji je poslužio sofisticiranoj morfometrijskoj analizi ponikava. Poligonalna struktura dobivena je iz digitalnog modela reljefa uz pomoć GIS hidroloških alata

    Megjegyzés a levelezési koncepció vitájához

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