Mutations disrupting the kinase domain of IKKα lead to immunodeficiency and immune dysregulation in humans.

IKKα, encoded by CHUK, is crucial in the non-canonical NF-κB pathway and part of the IKK complex activating the canonical pathway alongside IKKβ. The absence of IKKα causes fetal encasement syndrome in humans, fatal in utero, while an impaired IKKα-NIK interaction was reported in a single patient and causes combined immunodeficiency. Here, we describe compound heterozygous variants in the kinase domain of IKKα in a female patient with hypogammaglobulinemia, recurrent lung infections, and Hay-Wells syndrome-like features.

Robust Anti-Icing Surfaces Based on Dual Functionality─Microstructurally-Induced Ice Shedding with Superimposed Nanostructurally-Enhanced Water Shedding.

Research into anti-icing surfaces often conflates the two separate problems of ice accumulation: water adhesion and ice adhesion. The body feathers of perpetually ice-free penguins are very good natural examples of anti-icing surfaces, which use two different mitigation strategies for the two disparate problems. Herein, we mimic the form of the feather's wire-like structure, which is decorated with superimposed nanogrooves by laser micromachining fine woven wire cloths.

Second-order nonlinear optical coefficients of the monoclinic crystal BaGaSe.

We characterize the magnitude and relative signs of the second-order nonlinear optical coefficients of the biaxial crystal BaGaSe (BGSe) combining measurements of phase-matched and non-phase-matched second-harmonic generation at different wavelengths using slabs and a sphere.