Investigation of the SH3BP2 Gene Mutation in Cherubism

Cherubism is a rare developmental lesion of the jaw that is generally inherited as an autosomal dominant trait. Recent studies have revealed point mutations in the SH3BP2 gene in cherubism patients. In this study, we examined a 6-year-old Korean boy and his family. We found a Pro418Arg mutation in the SH3BP2 gene of the patient and his mother. A father and his 30-month-old younger brother had no mutations. Immunohistochemically, the multinucleated giant cells proved positive for CD68 and tartrate-resistant acid phosphatase (TRAP). Numerous spindle-shaped stromal cells expressed a ligand for receptor activator of nuclear factor kB (RANKL), but not in multinucleated giant cells. These results provide evidence that RANKL plays a critical role in the differentiation of osteoclast precursor cells to multinucleated giant cells in cherubism. Additionally, genetic analysis may be a useful method for differentiation of cherubism.</p

Effects of Nd-doping on the structural, electrical, and multiferroic properties of Bi7Fe3Ti3O21 thin films

Aurivillius-phase six-layered Bi7Fe3Ti3O21 (BFTO21) and Nd-doped Bi6.4Nd0.6Fe3Ti3O21 (BNdFTO21) thin films were prepared on Pt(111)/Ti/SiO2/Si(100) substrates by using a chemical solution deposition method in order to investigate their structural, electrical, and multiferroic properties. Doping the Bi sites of the BFTO21 with Nd-ions led to remarkable improvements in the electrical and the multiferroic properties. The electrical study of the BNdFTO21 thin film showed a low leakage current density of 4.38 × 10-6 A/cm at an applied electric field of 100 kV/cm, which was about one order of magnitude lower than that of the BFTO21 thin film. The ferroelectric P - E hysteresis loop of the BNdFTO21 thin film exhibited a large remnant polarization (2Pr) of 24 μC/cm2 and a low coercive electric field (2Ec) of 154 kV/cm at an applied electric field of 239 kV/cm. Furthermore, the magnetization and the coercive magnetic field that were observed for the BNdFTO21 thin film at room temperature were drastically enhanced compared to those observed for the BFTO21 thin film