Engineering atomic and molecular nanostructures at surfaces

The fabrication methods of the microelectronics industry have been refined to produce ever smaller devices, but will soon reach their fundamental limits. A promising alternative route to even smaller functional systems with nanometre dimensions is the autonomous ordering and assembly of atoms and molecules on atomically well-defined surfaces. This approach combines ease of fabrication with exquisite control over the shape, composition and mesoscale organization of the surface structures formed. Once the mechanisms controlling the self-ordering phenomena are fully understood, the self-assembly and growth processes can be steered to create a wide range of surface nanostructures from metallic, semiconducting and molecular materials.     

Mutations in SIL1 cause Marinesco-Sjögren syndrome, a cerebellar ataxia with cataract and myopathy

SIL1 (also called BAP) acts as a nucleotide exchange factor for the Hsp70 chaperone BiP (also called GRP78), which is a key regulator of the main functions of the endoplasmic reticulum. We found nine distinct mutations that would disrupt the SIL1 protein in individuals with Marinesco-Sj?gren syndrome, an autosomal recessive cerebellar ataxia complicated by cataracts, developmental delay and myopathy. Identification of SIL1 mutations implicates Marinesco-Sj?gren syndrome as a disease of endoplasmic reticulum dysfunction and suggests a role for this organelle in multisystem disorders.     

TNF-mediated inflammatory skin disease in mice with epidermis-specific deletion of IKK2

The I kappa B kinase (IKK), consisting of the IKK1 and IKK2 catalytic subunits and the NEMO (also known as IKK gamma) regulatory subunit, phosphorylates I kappa B proteins, targeting them for degradation and thus inducing activation of NF-kappa B (reviewed in refs 1, 2). IKK2 and NEMO are necessary for NF-kappa B activation through pro-inflammatory signals. IKK1 seems to be dispensable for this function but controls epidermal differentiation independently of NF-kappa B. Previous studies suggested that NF-kappa B has a function in the growth regulation of epidermal keratinocytes. Mice lacking RelB or I kappa B alpha, as well as both mice and humans with heterozygous NEMO mutations, develop skin lesions. However, the function of NF-kappa B in the epidermis remains unclear. Here we used Cre/loxP-mediated gene targeting to investigate the function of IKK2 specifically in epidermal keratinocytes. IKK2 deficiency inhibits NF-kappa B activation, but does not lead to cell-autonomous hyperproliferation or impaired differentiation of keratinocytes. Mice with epidermis-specific deletion of IKK2 develop a severe inflammatory skin disease, which is caused by a tumour necrosis factor-mediated, alpha beta T-cell-independent inflammatory response that develops in the skin shortly after birth. Our results suggest that the critical function of IKK2-mediated NF-kappa B activity in epidermal keratinocytes is to regulate mechanisms that maintain the immune homeostasis of the skin.     

Acidity: Modes of characterization and quantification

This paper provides an account of early historical developments in the characterization and quantification of acidity, which may be considered preliminary steps leading to the measurement of acidity. In this “pre-history” of acidity measurement, emphasis is laid on the relative independence of the rich empirical knowledge about acids from theories of acidity. Many attempts were made to compare and assess the strengths of various acids, based on concrete laboratory operations. However, at least until the arrival of the pH measure, the quantification attempts failed to produce anything qualifying as a measurement scale of a recognizable type. It is doubtful whether even pH qualifies as a true measure of acidity, when the full meaning of acidity is taken into account.     

A young source for the Hawaiian plume

Recycling of oceanic crust through subduction, mantle upwelling, and remelting in mantle plumes is a widely accepted mechanism to explain ocean island volcanism. The timescale of this recycling is important to our understanding of mantle circulation rates. Correlations of uranogenic lead isotopes in lavas from ocean islands such as Hawaii or Iceland, when interpreted as model isochrons, have yielded source differentiation ages between 1 and 2.5?billion years (Gyr). However, if such correlations are produced by mixing of unrelated mantle components they will have no direct age significance. Re-Os decay model ages take into account the mixing of sources with different histories, but they depend on the assumed initial Re/Os ratio of the subducted crust, which is poorly constrained because of the high mobility of rhenium during subduction. Here we report the first data on (87)Sr/(86)Sr ratios for 138 melt inclusions in olivine phenocrysts from lavas of Mauna Loa shield volcano, Hawaii, indicating enormous mantle source heterogeneity. We show that highly radiogenic strontium in severely rubidium-depleted melt inclusions matches the isotopic composition of 200-650-Myr-old sea water. We infer that such sea water must have contaminated the Mauna Loa source rock, before subduction, imparting a unique 'time stamp' on this source. Small amounts of seawater-derived strontium in plume sources may be common but can be identified clearly only in ultra-depleted melts originating from generally highly (incompatible-element) depleted source components. The presence of 200-650-Myr-old oceanic crust in the source of Hawaiian lavas implies a timescale of general mantle circulation with an average rate of about 2 (±1)?cm?yr(-1), much faster than previously thought.     

Forecasting quality and information

It is assumed that demand for information that subjectively appears to be relevant for forecasting improves forecasting quality. To study this hypothesis a number of forecasting experiments were conducted. Fifty managers from the housing business, from banking, and from a research institution were asked to forecast interest rates, using a Delphi process. They communicated via a computer system, and, to support their judgements, they had access to a data bank that was stored in the same system. Their communication with the system was automatically recorded. Part of the data collected in these experiments is used to study the existence of a relationship between information activities and forecasting results. A weak positive relationship is found if non-linear functions are tested, where information demand is corrected by those data retrievals that seem to have resulted from an inability to handle the information system. For further research a more general, albeit less informative, Boolean model is suggested.