A set of glutamylases and deglutamylases controls levels of tubulin polyglutamylation, a prominent post-translational modification of neuronal microtubules. Defective tubulin polyglutamylation was first linked to neurodegeneration in the Purkinje cell degeneration (pcd) mouse, which lacks deglutamylase CCP1, displays massive cerebellar atrophy, and accumulates abnormally glutamylated tubulin in degenerating neurons. We found biallelic rare and damaging variants in the gene encoding CCP1 in 13 individuals with infantile-onset neurodegeneration and confirmed the absence of functional CCP1 along with dysregulated tubulin polyglutamylation. The human disease mainly affected the cerebellum, spinal motor neurons, and peripheral nerves. We also demonstrate previously unrecognized peripheral nerve and spinal motor neuron degeneration in pcd mice, which thus recapitulated key features of the human disease. Our findings link human neurodegeneration to tubulin polyglutamylation, entailing this post-translational modification as a potential target for drug development for neurodegenerative disorders.

Although graphene-based thermal interface materials (TIMs) have great potentials in removing excess heat generated during highly efficient running of electronic devices, their practical applications are usually limited by their unsatisfactory thermal conductions, which are mainly caused by unsatisfactory dispersion and distribution, low loading, and low quality of graphene sheets, as well as the thermal interfacial resistance between graphene sheets and polymer matrix. Herein, we develop vertically aligned graphene hybrid foams (GHFs) with high densities by hydrothermal reduction of graphene oxide in the presence of high-quality graphene nanoplatelets (GNPs) followed by air-drying. The reduced graphene oxide sheets play an important role in constructing a vertically aligned interconnection network for accommodating GNPs during the hydrothermal reduction process, while the incorporated GNPs not only make the thermal conductance network denser but also prevent excessive shrinkage of the foams during air-drying. More critically, graphitization of GHF at 2800 °C removes the residual oxygen-containing groups and heals the defects of their reduced graphene oxide component, leading to high-quality graphene foams. The resultant vertically aligned high-quality graphene porous architecture with high density as an ideal thermal conductance network of TIMs is highly efficient in improving the thermal conductivity of its epoxy composite, which exhibits an ultrahigh through-plane thermal conductivity of 35.5 W m K at a graphene loading of 19.0 vol %. The excellent thermally conductive performance makes the annealed GHF/epoxy composites suitable for the thermal management.

The power conversion efficiencies (PCEs) of the solar cells containing metal halide perovskites (MHPs) have rapidly increased and exceeded 25% during the past decade. The photovoltaic properties of these devices are extensively investigated in terms of their microstructures, environmental characteristics, and carrier dynamics, and the MHP structural evolution under high pressure is evaluated. In addition, the energy level structure, electron/hole dynamics, and optical/electronic properties of MHPs with anisotropic crystal structures are examined. However, the correlation between the structural anisotropy and material properties of these perovskites is rarely considered in the literature studies on their high‐pressure behavior. In this progress report, the optical/electronic properties of MHPs with anisotropic structures under thermal, mechanically imposed, and in‐service strains/stresses that have been previously neglected by researchers are summarized.

The γ-tubulin ring complex (γTuRC) is the primary microtubule nucleator in animal cells. NME7 possesses an intrinsic kinase activity that is involved in the stimulation of the γTuRC.