Mutations in the gene encoding the 3'-5' DNA exonuclease TREX1 cause Aicardi-Goutières syndrome at the AGS1 locus

Aicardi-Goutières syndrome (AGS) presents as a severe neurological brain disease and is a genetic mimic of the sequelae of transplacentally acquired viral infection. Evidence exists for a perturbation of innate immunity as a primary pathogenic event in the disease phenotype. Here, we show that TREX1, encoding the major mammalian 3' --> 5' DNA exonuclease, is the AGS1 gene, and AGS-causing mutations result in abrogation of TREX1 enzyme activity. Similar loss of function in the Trex1(-/-) mouse leads to an inflammatory phenotype. Our findings suggest an unanticipated role for TREX1 in processing or clearing anomalous DNA structures, failure of which results in the triggering of an abnormal innate immune response.     

An off-axis hydrothermal vent field near the Mid-Atlantic Ridge at 30 degrees N.

Evidence is growing that hydrothermal venting occurs not only along mid-ocean ridges but also on old regions of the oceanic crust away from spreading centres. Here we report the discovery of an extensive hydrothermal field at 30 degrees N near the eastern intersection of the Mid-Atlantic Ridge and the Atlantis fracture zone. The vent field--named 'Lost City'--is distinctly different from all other known sea-floor hydrothermal fields in that it is located on 1.5-Myr-old crust, nearly 15 km from the spreading axis, and may be driven by the heat of exothermic serpentinization reactions between sea water and mantle rocks. It is located on a dome-like massif and is dominated by steep-sided carbonate chimneys, rather than the sulphide structures typical of 'black smoker' hydrothermal fields. We found that vent fluids are relatively cool (40-75 degrees C) and alkaline (pH 9.0-9.8), supporting dense microbial communities that include anaerobic thermophiles. Because the geological characteristics of the Atlantis massif are similar to numerous areas of old crust along the Mid-Atlantic, Indian and Arctic ridges, these results indicate that a much larger portion of the oceanic crust may support hydrothermal activity and microbial life than previously thought.     

Long-term eruptive activity at a submarine arc volcano

Three-quarters of the Earth's volcanic activity is submarine, located mostly along the mid-ocean ridges, with the remainder along intraoceanic arcs and hotspots at depths varying from greater than 4,000 m to near the sea surface. Most observations and sampling of submarine eruptions have been indirect, made from surface vessels or made after the fact. We describe here direct observations and sampling of an eruption at a submarine arc volcano named NW Rota-1, located 60 km northwest of the island of Rota (Commonwealth of the Northern Mariana Islands). We observed a pulsating plume permeated with droplets of molten sulphur disgorging volcanic ash and lapilli from a 15-m diameter pit in March 2004 and again in October 2005 near the summit of the volcano at a water depth of 555 m (depth in 2004). A turbid layer found on the flanks of the volcano (in 2004) at depths from 700 m to more than 1,400 m was probably formed by mass-wasting events related to the eruption. Long-term eruptive activity has produced an unusual chemical environment and a very unstable benthic habitat exploited by only a few mobile decapod species. Such conditions are perhaps distinctive of active arc and hotspot volcanoes.     

Association of coronavirus infection with hemorrhagic entercolitis in newborn infants

The existence of a widely spread corona virus epidemic enabled us to associate this infection with necrotizing hemorrhagic enterocolitis in newborn infants. Virus is detected in the stools by direct electron microscopic examination. Virions are found agglutinated in the gut during convalescence, as they are by circulating antibodies. Arguments supporting a possible etiological role of this infection are given.