Mutations in SEC63 cause autosomal dominant polycystic liver disease

Mutations in PRKCSH, encoding the beta-subunit of glucosidase II, an N-linked glycan-processing enzyme in the endoplasmic reticulum (ER), cause autosomal dominant polycystic liver disease. We found that mutations in SEC63, encoding a component of the protein translocation machinery in the ER, also cause this disease. These findings are suggestive of a role for cotranslational protein-processing pathways in maintaining epithelial luminal structure and implicate noncilial ER proteins in human polycystic disease.     

Dislocation-driven surface dynamics on solids

Dislocations are line defects that bound plastically deformed regions in crystalline solids. Dislocations terminating on the surface of materials can strongly influence nanostructural and interfacial stability, mechanical properties, chemical reactions, transport phenomena, and other surface processes. While most theoretical and experimental studies have focused on dislocation motion in bulk solids under applied stress and step formation due to dislocations at surfaces during crystal growth, very little is known about the effects of dislocations on surface dynamics and morphological evolution. Here we investigate the near-equilibrium dynamics of surface-terminated dislocations using low-energy electron microscopy. We observe, in real time, the thermally driven nucleation and shape-preserving growth of spiral steps rotating at constant temperature-dependent angular velocities around cores of dislocations terminating on the (111) surface of TiN in the absence of applied external stress or net mass change. We attribute this phenomenon to point-defect migration from the bulk to the surface along dislocation lines. Our results demonstrate that dislocation-mediated surface roughening can occur even in the absence of deposition or evaporation, and provide fundamental insights into mechanisms controlling nanostructural stability.     

The interface between silicon and a high-k oxide

The ability of the semiconductor industry to continue scaling microelectronic devices to ever smaller dimensions (a trend known as Moore's Law) is limited by quantum mechanical effects: as the thickness of conventional silicon dioxide (SiO(2)) gate insulators is reduced to just a few atomic layers, electrons can tunnel directly through the films. Continued device scaling will therefore probably require the replacement of the insulator with high-dielectric-constant (high-k) oxides, to increase its thickness, thus preventing tunnelling currents while retaining the electronic properties of an ultrathin SiO(2) film. Ultimately, such insulators will require an atomically defined interface with silicon without an interfacial SiO(2) layer for optimal performance. Following the first reports of epitaxial growth of AO and ABO(3) compounds on silicon, the formation of an atomically abrupt crystalline interface between strontium titanate and silicon was demonstrated. However, the atomic structure proposed for this interface is questionable because it requires silicon atoms that have coordinations rarely found elsewhere in nature. Here we describe first-principles calculations of the formation of the interface between silicon and strontium titanate and its atomic structure. Our study shows that atomic control of the interfacial structure by altering the chemical environment can dramatically improve the electronic properties of the interface to meet technological requirements. The interface structure and its chemistry may provide guidance for the selection process of other high-k gate oxides and for controlling their growth.     

The fossil vertebrates from East Greenland and their zoological importance

Zusammenfassung Kurzer Bericht über die dänischen Expeditionen nach Ostgrönland (Lauge Koch) 1929–1957. Die zoologische Bedeutung des fossilen Vertebratenmaterials (über 10000 Exemplare) wird angedeutet. Neuere Ergebnisse über die Evolution der Tetrapodenzunge werden besonders im Zusammenhang mit Grönlandmaterial besprochen. Damit wird die Ansicht bestätigt, dass sich die Urodelen selbständig aus porolepiformen Crossopterygiern entwickelt haben, während Anuren, Sauropsiden und Säugetiere von Osteolepiformiern abzuleiten sind. Die Auswertung des umfangreichen Fossilmaterials ergibt eine diphyletische Herkunft der Tetrapoden.     

Thein vitro inhibition of pentobarbital metabolism by chlorpromazine

Riassunto Si è osservato che il pentobarbital alla concentrazione di 2 × 10^–4 M può inibire per il 44% il metabolismoin vitro della cloropromazina alla medesima concentrazione.Si è discussa quindi la possibilità di inibire il metabolismoin vitro di alcuni farmaci propria di altre sostanze che vengono metabolizzate in maniera del tutto simile da sistemi enzimatici a livello microsomale epatico.