Ellis-van Creveld Syndrome in Grey Alpine Cattle: Morphologic, Immunophenotypic, and Molecular Characterization

Ellis-van Creveld (EvC) syndrome is a human autosomal recessive disorder caused by a mutation in either the EVC or EVC2 gene, and presents with short limbs, polydactyly, and ectodermal and heart defects. The aim of this study was to understand the pathologic basis by which deletions in the EVC2 gene lead to chondrodysplastic dwarfism and to describe the morphologic, immunohistochemical, and molecular hallmarks of EvC syndrome in cattle. Five Grey Alpine calves, with a known mutation in the EVC2 gene, were autopsied. Immunohistochemistry was performed on bone using antibodies to collagen II, collagen X, sonic hedgehog, fibroblast growth factor 2, and Ki67. Reverse transcription polymerase chain reaction was performed to analyze EVC1 and EVC2 gene expression. Autopsy revealed long bones that were severely reduced in length, as well as genital and heart defects. Collagen II was detected in control calves in the resting, proliferative, and hypertrophic zones and in the primary and secondary spongiosa, with a loss of labeling in the resting zone of 2 dwarfs. Collagen X was expressed in hypertrophic zone in the controls but was absent in the EvC cases. In affected calves and controls, sonic hedgehog labeled hypertrophic chondrocytes and primary and secondary spongiosa similarly. FGF2 was expressed in chondrocytes of all growth plate zones in the control calves but was lost in most EvC cases. The Ki67 index was lower in cases compared with controls. EVC and EVC2 transcripts were detected. Our data suggest that EvC syndrome of Grey Alpine cattle is a disorder of chondrocyte differentiation, with accelerated differentiation and premature hypertrophy of chondrocytes, and could be a spontaneous model for the equivalent human disease.

An inter-regional assessment of concentrations and δ13C values of methane and dissolved inorganic carbon in small European lakes

Methane (CH4) and carbon dioxide emissions from lakes are relevant for assessing the greenhouse gas output of wetlands. However, only few standardized datasets describe concentrations of these gases in lakes across different geographical regions. We studied concentrations and stable carbon isotopic composition (δ13C) of CH4 and dissolved inorganic carbon (DIC) in 32 small lakes from Finland, Sweden, Germany, the Netherlands, and Switzerland in late summer. Higher concentrations and δ13C values of DIC were observed in calcareous lakes than in lakes on non-calcareous areas. In stratified lakes, δ13C values of DIC were generally lower in the hypolimnion due to the degradation of organic matter (OM). Unexpectedly, increased δ13C values of DIC were registered above the sediment in several lakes. This may reflect carbonate dissolution in calcareous lakes or methanogenesis in deepwater layers or in the sediments. Surface water CH4 concentrations were generally higher in western and central European lakes than in Fennoscandian lakes, possibly due to higher CH4 production in the littoral sediments and lateral transport, whereas CH4 concentrations in the hypolimnion did not differ significantly between the regions. The δ13C values of CH4 in the sediment suggest that δ13C values of biogenic CH4 are not necessarily linked to δ13C values of sedimentary OM but may be strongly influenced by OM quality and methanogenic pathway. Our study suggests that CH4 and DIC cycling in small lakes differ between geographical regions and that this should be taken into account when regional studies on greenhouse gas emissions are upscaled to inter-regional scales.

Imperial Eyes. Van Dycks Porträtmalerei als globale Technologie

Zu Beginn des 17. Jahrhunderts hatte sich die Malerei in Antwerpen vor allem durch Rubens als Medium einer zunehmend global verbundenen Welt etabliert. Doch auch zahlreiche von Van Dycks Porträts sind Zeugnisse eines oft hochgradig ambivalenten Kosmopolitismus. Neben Gemälden von international agierenden Antwerpener Händlern und Gelehrten fertigte der Künstler auch in Genua und England Porträts der adligen Elite an, in welchen sich deren globale Erfahrungen und Ambitionen spiegelten. Van Dyck reagierte in der Inszenierung der Marchesa Elena Grimaldi Cattaneo, mit einem schwarzen Sklaven, wie in der des Earl of Denbigh, zwischen einheimischen und exotischen Elementen, auf spezifische Anforderungen an das repräsentative Porträt im Zeitalter des disenclavement. Diese Anforderungen werden besonders deutlich in dem für eine geplante Expedition nach Madagaskar entstandenen Porträt des Earls of Arundel und dessen Frau Alethea Talbot. Das Gemälde verbindet Elemente herrschaftlicher, merkantilistischer und intellektueller Selbststilisierung mit einer Reduktion auf wenige, innovative Elemente. Die majestätische Inszenierung des Paars ist nur in einem globalen Kontext zu erklären, so dass sich die Frage nach der Funktion des in mehreren Werkstattkopien erhaltenen Gemäldes stellt. Hatte Van Dyck in seiner Komposition eine Lösung gefunden, die im Kontext des englischen Hofes, möglicherweise als Teil eines Festes, wie auch für potentielle Investoren, sowie die verarmte Landbevölkerung, die in der Indentur ihre einzige Hoffnung sah, gleichermaßen überzeugend wirkte? Die Hybris in dem überlebensgroßen Porträts des Paares, das mit leuchtend heller Haut, in kostbar glänzende Stoffe gekleidet und mit modernen Messinstrumenten in den Händen dargestellt ist, lässt es als ideales Medium der globalen Ambitionen des Paars erscheinen.