Making ordered nanostructures in polymers and their thin films is an important technique to produce functional materials. Herein, we report instant yet precise self-assembly systems of amphiphilic random copolymers to build multilayered lamellar structures in bulk materials and thin films. Random copolymers bearing octadecyl groups and hydroxyethyl groups induced crystallization-driven microphase separation via simple evaporation from the solutions to form lamellar structures in the solid state. The domain spacing was controlled in the range between 3.1 and 4.2 nm at the 0.1 nm level by tuning copolymer composition. Interestingly, just by spin-coating the polymer solutions onto silicon substrates, the copolymers autonomously formed thin films consisting of multilayered lamellar structures, where amorphous/hydrophilic parts and crystalline octadecyl domains are alternatingly layered from a silicon substrate to the air/polymer interface at regular intervals. The lamellar domain spacing was tunable by selecting hydrophilic pendants.

Summary C21ORF2 and NEK1 have been identified as amyotrophic lateral sclerosis (ALS)-associated genes. Both genes are also mutated in certain ciliopathies, suggesting that they might contribute to the same signaling pathways. Here we show that FBXO3, the substrate receptor of an SCF ubiquitin ligase complex, binds and ubiquitylates C21ORF2, thereby targeting it for proteasomal degradation. C21ORF2 stabilizes the kinase NEK1, with the result that loss of FBXO3 stabilizes not only C21ORF2 but also NEK1. Conversely, NEK1-mediated phosphorylation stabilizes C21ORF2 by attenuating its interaction with FBXO3. We found that the ALS-associated V58L mutant of C21ORF2 is more susceptible to phosphorylation by NEK1, with the result that it is not ubiquitylated by FBXO3 and therefore accumulates together with NEK1. Expression of C21ORF2(V58L) in motor neurons induced from mouse embryonic stem cells impaired neurite outgrowth. We suggest that inhibition of NEK1 activity is a potential therapeutic approach to ALS associated with C21ORF2 mutation.

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The first stable neutral stannaaromatic compound, 2-stannanaphthalene , was synthesized by taking advantage of an extremely bulky and efficient steric protection group, 2,4,6-tris[bis(trimethylsilyl)methyl]phenyl (Tbt). The molecular structure and aromaticity of were discussed on the basis of X-ray crystallographic analysis, NMR, UV-vis, and Raman spectroscopy, cyclic voltammetry, and theoretical calculations. 2-Germanaphthalene , which has a framework similar to that of , was synthesized for comparison, and systematic elucidation was made for the properties of 2-metallanaphthalene systems containing a heavier group 14 element (Si, Ge, or Sn).

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