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An SNP array-based genome-wide linkage analysis is conducted to explain the molecular basis and symptoms of the adducted thumb-clubfoot syndrome. The results demonstrate that a particular defect of dermatan sulfate biosynthesis is the main factor that leads to the onset of the autosomal-recessive disorder.

A genome-wide SNP scan is used to study the characteristics and causes of the syndromic form of the autosomal-recessive congenital sodium diarrhea (CSD). The classic CSD is shown to be distinct from the syndromic CSD, which is shown to be caused by the mutations caused in SPINT2.

Results of the particle flux studies using sediment traps in the northeast Atlantic Ocean at 33 Degree N between summer 1993 and summer 2001 are presented. The results suggest that the interannual differences in the observed particle flux are a direct consequence of the variability of phytoplankton biomass and primary production in the euphotic zone in this region.

[beta]-Catenin is an essential component of the canonical Wnt signaling system that controls decisive steps in development. We employed here two conditional/3-catenin mutant alleles to alter [beta]-catenin signaling in the central nervous system of mice: one allele to ablate [beta]-catenin and the second allele to express a constitutively active [beta]-catenin. The tissue mass of the spinal cord and brain is reduced after ablation of [beta]-catenin, and the neuronal precursor population is not maintained. In contrast, the spinal cord and brain of mice that express activated [beta]-catenin is much enlarged in mass, and the neuronal precursor population is increased in size. [beta]-Catenin signals are thus essential for the maintenance of proliferation of neuronal progenitors, controlling the size of the progenitor pool, and impinging on the decision of neuronal progenitors to proliferate or to differentiate. Keywords: Wnt signaling; [beta]-Catenin; Neural stem cells; Neuronal progenitors; Progenitor pool; Proliferation versus differentiation

Pax1 and Pax9 represent a subfamily of paired-box-containing genes. In vertebrates, Pax1 and Pax9 transcripts have been found specifically in mesodermal tissues and the pharyngeal endoderm. Pax1 expression in the sclerotomes has been shown to be indispensable for proper formation of the axial skeleton, but expression of Pax1 in the endoderm has not been studied in detail. We have cloned the chick homologue of the murine Pax9 gene. Our results show that transcripts of Pax1 and Pax9 are first detectable in the prospective foregut endoderm of headfold-stage avian embryos. Endodermal expression correlates with the highly proliferative zones of the folding foregut and evaginating pharyngeal pouches. In later stages, Pax1 and Pax9 are expressed in overlapping but distinct patterns within the developing sclerotomes and limb buds. From grafting experiments we conclude that activation of pharyngeal Pax1 and Pax9 expression is an intrinsic property of the endoderm, not requiring midline structures or head mesoderm. In contrast, notochord is required to induce Pax1 in competent sclerotomes. Here we show that in vitro there is a cranio-caudal gradient of inductive capacity in the notochord. This coincides with the graded expression of Pax1 and Pax9 along the cranio-caudal axis in 2- to 3-day-old embryos. Furthermore, paraxial head mesoderm shows no competence to express Pax1. Finally, in vitro we find counteracting influences on notochord signaling by lateral tissues (lateral plate, intermediate mesoderm), leading to an inhibition of Sonic hedgehog (Shh) expression in notochord and floor plate, as well as Pax1 and Pax9 expression in sclerotomes. Taken together, our results demonstrate that different mechanisms regulate expression of Pax1 and Pax9 in foregut and sclerotome, but suggest a common function for both genes in the two tissues that is promoting proliferation and preventing fusion of neighboring blastemas.

Limb muscle formation involves invasion of the limb bud mesoderm by myogenic precursor cells from the dermomyotomes at limb bud level. Directed cell migration, homing, and differentiation of myogenic cells are controlled by the stationary cells of the limb bud mesoderm. At the level of the extracellular matrix, the molecular basis of migration control has been suggested to be exerted by the distribution of hyaluronan. Here, we demonstrate that N-cadherin-mediated interactions play a role at cell-membrane level in myoblast distribution and differentiation. N-cadherin is strongly expressed by myogenic cells in the chick limb bud and more moderately expressed by stationary mesodermal cells in the myogenic zones and progress zone. After in vivo injection of antibodies and Fab-fragments against the homophilic binding site of N-cadherin into the wing bud mesoderm, aggregates of myoblasts are found predominantly in the dorsal myogenic zone 36 hr after injection apparently due to immobilization. In the same position, areas of myf-5-positive cells are also observed. In injected limb buds, Pax-3-positive cells are less evenly distributed than in uninjected limbs. They are found to spread up to the epidermis and also form loosely arranged aggregates. After prolonged reincubation periods, injected limbs show ectopic myoblasts that are rich in desmin and areas of strongly desmin-expressing myoblasts within muscle blastemas. These effects were not observed after application of antibodies against other parts of the N-cadherin molecule. We conclude that N-cadherin is involved in myoblast migration in the limb buds via homophilic interactions and that it plays a role in signal transduction during myogenesis.

The advanced MKIIIB-conductivity-temperature-depth profiler is usable for hydrographic measurements. The profiler determines the physical parameters of sea water, temperature, pressure and electrical conductivity. The parameter sensors have bit discontinuities which are difficult to identify by the available dead weight testers. For better efficiency of these instruments, a - 3 degree celsius output is necessary.

The transport variability of the Eastern Boundary Current of the North Atlantic subtropical Gyre is described using observations from a four-year current meter mooring at 28 degree 44'N, 13 degree 28'W in the Lanzarote passage. A consistent northward transport is observed every autumn, which may be related with the eastern boundary upwelling dynamics.

Due to the implementation of oral rabies vaccination (ORV) programmes, the European Union (EU) is becoming progressively free of red fox (Vulpes vulpes)-mediated rabies. Over the past three decades, the incidence of rabies had decreased substantially and vast areas of Western and Central Europe have been freed from rabies using this method of controlling an infectious disease in wildlife. Since rabies control is a top priority in the EU, the disease is expected to be eliminated from the animal source in the near future. While responsible authorities may consider the mission of eliminating fox rabies from the EU almost accomplished, there are still issues to be dealt with and challenges to be met that have not yet been in the focus of attention, but could jeopardise the ultimate goal. Among them are increasing illegal movements of animals, maintaining funding support for vaccination campaigns, devising alternative vaccine strategies in neighbouring Eastern European countries and the expanding distribution range of several potential rabies reservoir species in Europe. [ABSTRACT FROM AUTHOR]

Abstract: A series of dendritic DTPA-derived ligands and their corresponding gadolinium(III) complexes have been prepared and their spreading behaviour at the air–water interface has been investigated. The results show that these amphiphiles have the prerequisite qualities to form stable and reversible Langmuir monolayers opening the road to novel magnetic organic films. [Copyright &y& Elsevier]

Focuses on the structure, regulation and selective inhibition of the type four cyclic adenosine monophosphate (cAMP) subtypes. Chromosomal localization and genomic organization; Transcriptional regulation and phosphorylation of phosphodiesterases (PDE); Molecular structure and enzyme activity; Distribution and expression of human PDE4 subtypes; Selective inhibition of human PDE4 subtypes; Conclusions.

• Policy narratives frame the Batwa people as either 'forest guardians' or 'forest destroyers' in Kahuzi-Biega National Park. • Yet, historical survey data shows armed conflict impacts large mammal populations more than the presence of Batwa. • Furthermore, satellite imagery shows significant tree cover loss in areas of the park reoccupied by Batwa since 2018. • While certain Batwa have contributed to this deforestation, they are but one minor player in a wider extractive dynamic. • Indigenous peoples' agency should be seen as existing in complex dialectical interplay with broader structural circumstances. This paper makes a significant empirical contribution to our understanding of the complex relations between indigenous people and nature. It builds on the literature on environmental narratives to show how for some policy actors, indigenous populations are seen as 'forest destroyers', and for others as 'forest guardians'. It argues that these narratives are based on ideal-type constructions, which frame indigenous agency as a central defence against or factor in environmental destruction. By doing so, they rationalize different roles for the state and indigenous peoples in conservation governance. On a surface level, the narratives appear as competing and incompatible. Yet, on closer inspection, they are stabilized within and reinforce a shared common sense: namely, that the fate of nature ultimately hinges upon indigenous peoples. Through an in-depth study of an indigenous group known as the Batwa in eastern Democratic Republic of Congo's (DRC) Kahuzi-Biega National Park, the paper challenges this viewpoint. Using both qualitative and quantitative data, including satellite imagery, it shows how the ideal-type narratives ultimately divert attention from a broader political economy of violent resource extraction, which is fundamental. In doing so, they account for policies that fail people and nature. [ABSTRACT FROM AUTHOR]