Mutations in genes encoding ribonuclease H2 subunits cause Aicardi-Goutières syndrome and mimic congenital viral brain infection

Aicardi-Goutières syndrome (AGS) is an autosomal recessive neurological disorder, the clinical and immunological features of which parallel those of congenital viral infection. Here we define the composition of the human ribonuclease H2 enzyme complex and show that AGS can result from mutations in the genes encoding any one of its three subunits. Our findings demonstrate a role for ribonuclease H in human neurological disease and suggest an unanticipated relationship between ribonuclease H2 and the antiviral immune response that warrants further investigation.     

Ultrafast holographic nanopatterning of biocatalytically formed silica

Diatoms are of interest to the materials research community because of their ability to create highly complex and intricate silica structures under physiological conditions: what these single-cell organisms accomplish so elegantly in nature requires extreme laboratory conditions to duplicate-this is true for even the simplest of structures. Following the identification of polycationic peptides from the diatom Cylindrotheca fusiformis, simple silica nanospheres can now be synthesized in vitro from silanes at nearly neutral pH and at ambient temperatures and pressures. Here we describe a method for creating a hybrid organic/inorganic ordered nanostructure of silica spheres through the incorporation of a polycationic peptide (derived from the C. fusiformis silaffin-1 protein) into a polymer hologram created by two-photon-induced photopolymerization. When these peptide nanopatterned holographic structures are exposed to a silicic acid, an ordered array of silica nanospheres is deposited onto the clear polymer substrate. These structures exhibit a nearly fifty-fold increase in diffraction efficiency over a comparable polymer hologram without silica. This approach, combining the ease of processability of an organic polymer with the improved mechanical and optical properties of an inorganic material, could be of practical use for the fabrication of photonic devices.