[Clinton] Larsen, saut en hauteur [vainqueur des Jeux interalliés avec 1m86, stade Pershing, le 4 juillet 1919] : [photographie de presse] / [Agence Rol]

Référence bibliographique : Rol, 54703

Congenital joint dislocations caused by carbohydrate sulfotransferase 3 deficiency in recessive Larsen syndrome and humero-spinal dysostosis

Deficiency of carbohydrate sulfotransferase 3 (CHST3; also known as chondroitin-6-sulfotransferase) has been reported in a single kindred so far and in association with a phenotype of severe chondrodysplasia with progressive spinal involvement. We report eight CHST3 mutations in six unrelated individuals who presented at birth with congenital joint dislocations. These patients had been given a diagnosis of either Larsen syndrome (three individuals) or humero-spinal dysostosis (three individuals), and their clinical features included congenital dislocation of the knees, elbow joint dysplasia with subluxation and limited extension, hip dysplasia or dislocation, clubfoot, short stature, and kyphoscoliosis developing in late childhood. Analysis of chondroitin sulfate proteoglycans in dermal fibroblasts showed markedly decreased 6-O-sulfation but enhanced 4-O-sulfation, confirming functional impairment of CHST3 and distinguishing them from diastrophic dysplasia sulphate transporter (DTDST)-deficient cells. These observations provide a molecular basis for recessive Larsen syndrome and indicate that recessive Larsen syndrome, humero-spinal dysostosis, and spondyloepiphyseal dysplasia Omani type form a phenotypic spectrum.

Foehn jets over the Larsen C ice shelf, Antarctica

Previously unknown foehn jets have been identified to the east of the Antarctic Peninsula (AP) above the Larsen C Ice Shelf. These jets have major implications for the east coast of the AP, a region of rapid climatic warming and where two large sections of ice shelf have collapsed in recent years. During three foehn events across the AP, leeside warming and drying is seen in new aircraft observations and simulated well by the Met Office Unified Model (MetUM) at ∼1.5 km grid spacing. In case A, weak southwesterly flow and an elevated upwind inversion characterise a highly nonlinear flow regime with upwind flow blocking. In case C strong northwesterly winds characterise a relatively linear case with little upwind flow blocking. Case B resides somewhere between the two in flow regime linearity. The foehn jets – apparent in aircraft observations where available and MetUM simulations of all three cases – are mesoscale features (up to 60 km in width) originating from the mouths of leeside inlets. Through back trajectory analysis they are identified as a type of gap flow. In cases A and B the jets are distinct, being strongly accelerated relative to the background flow, and confined to low levels above the Larsen C Ice Shelf. They resemble the ‘shallow foehn’ of the Alps. Case C resembles a case of ‘deep foehn’, with the jets less distinct. The foehn jets are considerably cooler and moister relative to adjacent regions of calmer foehn air. This is due to a dampened foehn effect in the jet regions: in case A the jets have lower upwind source regions, and in the more linear case C there is less diabatic warming and precipitation along jet trajectories due to the reduced orographic uplift across the mountain passes.

Thyra Larsen als Salome, Szenenfoto, in: Richard Strauss: Salome

Signatur des Originals: S 36/F05131

Thyra Larsen als Salome, Rollenbild, Brustbild, in: Richard Strauss: Salome

Signatur des Originals: S 36/F05132

Thyra Larsen als Salome, Szenenfoto, in: Richard Strauss: Salome

Signatur des Originals: S 36/F05133

Thyra Larsen als Salome, Rollenbild, Brustbild, Profil, in: Richard Strauss: Salome

Signatur des Originals: S 36/F05134

Thyra Larsen als Salome, Rollenbild, Brustbild, in: Richard Strauss: Salome

Signatur des Originals: S 36/F05135

Viggo Larsen, Brustbild

Signatur des Originals: S 36/F05136

Under ice shelf photographs from Drescher Inlet (Riiser Larsen Ice Shelf) taken by seal mounted cameras during expedition DRE2003

While modern sampling techniques, such as autonomous underwater vehicles, are increasing our knowledge of the fauna beneath Antarctic sea ice of only a few meters in depth, greater sampling difficulties mean that little is known about the marine life underneath Antarctic ice shelves over 100 m thick. In this study, we present underwater images showing the underside of an Antarctic ice shelf covered by aggregated invertebrate communities, most likely cnidarians and isopods. These images, taken at an average depth of 145 m, were obtained with a digital still camera system attached to Weddell seals Leptonychotes weddellii foraging just beneath the ice shelf. Our observations indicate that, similar to the sea floor, ice shelves serve as an important habitat for a remarkable amount of marine invertebrate fauna in Antarctica.