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

Primary Candida guilliermondii Infection of the Knee in a Patient without Predisposing Factors

Isolated primary candidal infection of joint is extremely rare, with only a few reported cases. It occurs as a result of accidental implantations of fungus during traumatic procedures, such as surgery, and is usually reported in patients with predisposing factors such as immunosuppression, malignancy, and drug abuse. If left untreated, irreversible deformity and pain with severe osteoarticular destruction occur. Thus, early diagnosis and treatment are important. This paper presents a case of 72-year-old man with primary C. guilliermondii infection of knee joint without predisposing factors and previous traumatic procedures, who was misdiagnosed with advanced degenerative osteoarthritis. Our case is the second case of primary C. guilliermondii arthritis of knee to be reported in the English-language literature and the first to be successfully treated with total knee arthroplasty following IV amphotericin B and oral fluconazole. Primary candidal infection of joint is generally asymptomatic or involves only mild pain and swelling in the affected knee. Thus, although the majority of knee joint infections are of a pyogenic or tuberculous origin, if a patient complains of mild pain and swelling in the knee and has mild signs of infection, the possibility of fungal infection should be considered

Theory of magnetic field-induced metaelectric critical end point in BiMn2_2O5_5

A recent experiment on the multiferroic BiMn$_2$O$_5$ compound under a strong applied magnetic field revealed a rich phase diagram driven by the coupling of magnetic and charge (dipolar) degrees of freedom. Based on the exchange-striction mechanism, we propose here a theoretical model with the intent to capture the interplay of the spin and dipolar moments in the presence of a magnetic field in BiMn$_2$O$_5$. Experimentally observed behavior of the dielectric constants, magnetic susceptibility, and the polarization is, for the most part, reproduced by our model. The critical behavior observed near the polarization reversal $(P=0)$ point in the phase diagram is interpreted as arising from the proximity to the critical end point.Comment: Theory; relevant experiment uploaded as arXiv:0810.190

Design and Implementation of Storage System Using Byte-index Chunking Scheme

In this paper, we present an enhanced storage system that supports Byte-index chunking algorithm. The storage system aims to provide efficient data deduplication with high performance and to be performed in rapid time. We describe the overall procedure of Byteindex chunking based storage system including read/write procedure and how the system works. The key idea of Byte-index chunking is to adapt fixed-size block chunk scheme which are distributed to “Index-table ” by chunk’s both side boundary values. We have found that Byte-index chunking in storage system provides high performance compared with other chunking schemes. Experiments result shows that the storage system with Byte-index chunking compresses overall data with high deduplication capability and reduce the speed of file processing

Role of Matrix Metalloproteinase 3-mediated alpha-Synuclein Cleavage in Dopaminergic Cell Death

Evidence suggests that the C-terminal truncation of alpha-synuclein is equally important as aggregation of alpha-synuclein in Parkinson disease (PD). Our previous results showed that an endopeptidase, matrix metalloproteinase-3 (MMP3), was induced and activated in dopaminergic (DA) cells upon stress conditions. Here, we report that MMP3 cleaved alpha-synuclein in vitro and in vivo and that alpha-synuclein and MMP3 were co-localized in Lewy bodies (LB) in the postmortem brains of PD patients. Incubation of alpha-synuclein with the catalytic domain of MMP3 (cMMP3) resulted in generation of several peptides, and the peptide profiles of WT alpha-synuclein (WTsyn) and A53T mutant (A53Tsyn) were different. Combined analysis using mass spectrometry and N-terminal determination revealed that MMP3 generated C-terminally truncated peptides of amino acids 1-78, 1-91, and 1-93 and that A53Tsyn produced significantly higher quantities of these peptides. Similar sizes of peptides were detected in N27 DA cells under oxidative stress and RNA interference to knock down MMP3-attenuated peptide generation. Co-overexpression of cMMP3 with either WTsyn or A53Tsyn led to a reduction in Triton X-100-insoluble aggregates and an increase in protofibril-like small aggregates. In addition, overexpression of the 1-93-amino acid peptide in the substantia nigra led to DA neuronal loss without LB-like aggregate formation. The results strongly indicate that MMP3 digestion of alpha-synuclein in DA neurons plays a pivotal role in the progression of PD through modulation of alpha-synuclein in aggregation, LB formation, and neurotoxicity

Effects of pre-existing hydrogen to stress triaxiality and damage evolution on ultra high strength steel

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There are arbitrary large minimal 2-pinning configurations

International audienceWe characterize minimal configurations that pin a line in every $2$-plane

Simultaneous fabrication of line defects-embedded periodic lattice by topographically assisted holographic lithography

We have demonstrated simultaneous fabrication of designed defects within a periodic structure. For rapid fabrication of periodic structures incorporating nanoscale line-defects at large area, topographically assisted holographic lithography (TAHL) technique, combining the strength of hologram lithography and phase-shift interference, was proposed. Hot-embossing method generated the photoresist patterns with vertical side walls which enabled phase-shift mask effect at the edge of patterns. Embossing temperature and relief height were crucial parameters for the successful TAHL process. Periodic holes with a diameter of 600 nm at a 1 μm-pitch incorporating 250 nm wide line-defects were obtained simultaneously