An Amyotrophic Lateral Sclerosis-Associated Mutant of C21ORF2 Is Stabilized by NEK1-Mediated Hyperphosphorylation and the Inability to Bind FBXO3.

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.

T-cell/histiocyte-rich large B-cell lymphoma of the thyroid.

T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL) is a rare subtype of diffuse large B-cell lymphoma, and has been reported to mainly affect lymph nodes with advanced Ann Arbor stage. We present a 69-year-old woman with a past history of chronic thyroiditis, who had suffered from rapidly-growing thyroid tumor. No other lymph nodes swelling or extranodal lesions were detected.

Synthesis and properties of stable 2-metallanaphthalenes of heavier group 14 elements.

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).

Nonscanning measurements for determining in-plane mode shapes in piezoelectric devices with polished surfaces.

A nonmechanical scanning method has been developed for the visualization of the in-plane mode shapes of piezoelectric devices with polished surfaces. By taking into account the reflection versus laser-wavelength characteristics of the material of the electrodes, the in-plane motion can be measured even if the surface of the measurement plane is polished like a mirror. This method is based on laser speckle interference and two-dimensional correlation filtering that effectively enhance the mode-shape visualization for bulk and surface acoustic wave devices.

Radiation protection system for interventional procedures of the upper extremity: evaluation in a phantom model.

Purpose: To design a radiation protection system for interventional procedures of the upper extremity and to evaluate the effectiveness of the system.

Materials And Methods: The radiation protection system consisted of an image intensifier (I-I) hood and x-ray tube cover. The I-I hood encircled the I-I to protect against scattered radiation from the patient's upper extremity and the table.