Impact of claudin-10 deficiency on amelogenesis: Lesson from a HELIX tooth.

In epithelia, claudin proteins are important components of the tight junctions as they determine the permeability and specificity to ions of the paracellular pathway. Mutations in CLDN10 cause the rare autosomal recessive HELIX syndrome (Hypohidrosis, Electrolyte imbalance, Lacrimal gland dysfunction, Ichthyosis, and Xerostomia), in which patients display severe enamel wear. Here, we assess whether this enamel wear is caused by an innate fragility directly related to claudin-10 deficiency in addition to xerostomia. A third molar collected from a female HELIX patient was analyzed by a combination of microanatomical and physicochemical approaches (i.e., electron microscopy, elemental mapping, Raman microspectroscopy, and synchrotron-based X-ray fluorescence). The enamel morphology, formation time, organization, and microstructure appeared to be within the natural variability. However, we identified accentuated strontium variations within the HELIX enamel, with alternating enrichments and depletions following the direction of the periodical striae of Retzius. These markings were also present in dentin. These data suggest that the enamel wear associated with HELIX may not be related to a disruption of enamel microstructure but rather to xerostomia. However, the occurrence of events of strontium variations within dental tissues might indicate repeated episodes of worsening of the renal dysfunction that may require further investigations.

Unusual hydrogen implanted gold with lattice contraction at increased hydrogen content.

The experimental evidence for the contraction of volume of gold implanted with hydrogen at low doses is presented. The contraction of lattice upon the addition of other elements is very rare and extraordinary in the solid-state, not only for gold but also for many other solids. To explain the underlying physics, the pure kinetic theory of absorption is not adequate and the detailed interaction of hydrogen in the lattice needs to be clarified. Our analysis points to the importance of the formation of hydride bonds in a dynamic manner and explains why these bonds become weak at higher doses, leading to the inverse process of volume expansion frequently seen in metallic hydrogen containers.

Strain-dependent structure and Raman behaviours in the heavy-ion irradiated manganite at extreme low dose.

The microstrains in heavy-ion irradiated manganite LaMnO3 can be managed in linear response of irradiation dose, and the corresponding internal pressure up to 8 GPa can be induced by varying doses. The response of structure under stress is studied by means of Density Functional Theory and Lattice Dynamic Calculation. All obtained Raman scattering lines are discussed in details to shed light onto structural changes during ion implantation. There appears new resonance peak at around 550 cm-1, which splits from broad features in the spectra, and attributes to the anti-symmetric vibrations of O6 cages. The blue shift of this peak scales to ~2.4 cm-1 per 1 GPa of stress. Another strong feature showing considerable blue shift is seen in the vicinity of 640 cm-1 and corresponds to one of rhombohedral distortion related soft modes. A weak mode, not frequently reported, is seen at around 420 cm-1 and corresponds to translation-like motions of fixed O6 cages.