ラフォラ ビョウ ノ ノウ ビョウリ ケイセイ トジョウキ ラフォラ ショウタイ ノ ビサイ コウゾウ ヘンカ

Lafora disease（LD）is an autosomal recessively inheritable neurodegeneration due to glycogen metabolic disorder, characterized pathologically by the presence of Lafora bodies（LB）（called polyglucosan bodies; malformed insoluble glucose polymers）in CNS neurons and cells in many other organs. Recently, it has become evident that about 90% of cases of LD are caused by mutations ineither the EPM2A（laforin）or the EPM2B（malin）gene. Few previous EM studies have observed LB in the developing phase in detail. This study aimed at obtaining key findings that may reveal molecular events involved in the disease mechanisms underlying the morphology, by EM observations on LB inbrain tissue from a previously reported case of LD. The observations revealed that the two main components of LB, i.e. poorly branched, irregular fine filaments and amorphous small dense granules, were distributed sparsely in the LB in the early phase, whereas in the developing phase the fine filaments were innumerable together with a substantial number of dense granules and polysomes along with some lysosomes and no apparent autophagosomes. They intermingled and formed LB. In the developed phase, cored LB consisted of aggregates of dense granules forming the core and radially arranged filaments forming the outer rim. Recently, it has been reported that the lack of laforin-malin complexes causes the dysfunction of autophagy, which plays a primary role in the LB formation. Thelack of Laforin-malin complexes and impaired autophagy for aggresome clearance were discussed. In conclusion, the fine structural changes of LB in the developing phase could be the key findings that link to the molecular mechanisms of not only LB but also LD

Operative approach for multiple primary lung carcinomas

AbstractOf 908 patients who underwent operation for primary lung cancer between January 1985 and June 1996, we considered 57 (6.3%) to have a second primary lung cancer, which was synchronous in 28 cases (3.1%) and metachronous in 29 cases (3.2%). Five-year survival for patients with synchronous and metachronous disease from initial treatment of cancer was 70.3% and 66.0%, respectively. Survival after the development of a metachronous lesion was 32.9% at 5 years. Sixteen of the synchronous second tumors (57%) were detected on preoperative radiography or bronchoscopy and 11 (39%) at the time of operation. Survival of patients at stage I or II from treatment of a synchronous lesion (p = 0.002) and of a metachronous second lesion (p = 0.028) was significantly better compared with those at stage III or IV. Therefore it is important to carefully examine a synchronous lesion before and during the operation of a primary lung cancer and to perform close follow-up surveillance for early detection of a metachronous lesion. In treating multiple lung carcinomas consideration should always be given to performing precise staging, aggressive operative approach for early stage, and oncologically sound parenchymal sparing procedures. (J Thorac Cardiovasc Surg 1998;115:836-40

Glycosylation reactions mediated by hypervalent iodine : application to the synthesis of nucleosides and carbohydrates

To synthesize nucleoside and oligosaccharide derivatives, we often use a glycosylation reaction to form a glycoside bond. Coupling reactions between a nucleobase and a sugar donor in the former case, and the reaction between an acceptor and a sugar donor of in the latter are carried out in the presence of an appropriate activator. As an activator of the glycosylation, a combination of a Lewis acid catalyst and a hypervalent iodine was developed for synthesizing 4’-thionucleosides, which could be applied for the synthesis of 4’-selenonucleosides as well. The extension of hypervalent iodine-mediated glycosylation allowed us to couple a nucleobase with cyclic allylsilanes and glycal derivatives to yield carbocyclic nucleosides and 2’,3’-unsaturated nucleosides, respectively. In addition, the combination of hypervalent iodine and Lewis acid could be used for the glycosylation of glycals and thioglycosides to produce disaccharides. In this paper, we review the use of hypervalent iodine-mediated glycosylation reactions for the synthesis of nucleosides and oligosaccharide derivatives

HEALTH ASPECTS OF KARATE AS PHYSICAL EDUCATION AND EXTRACURRICULAR ACTIVITY

The present study examined energy expenditure, metabolic equivalent (MET) intensities of karate exercises for health promotion, and bone properties of karate practitioners to examine the health aspects of karate as physical education and an extracurricular activity. The mean energy expenditure following a 70-minute karate practice was 563 kcal for men and 268 kcal for women. The calculated mean MET intensities resulting from the 70-minute karate practice were 7.9 METs for men and 5.2 METs for women. The mean MET intensities of all exercises for men and women were above 3 METs which is defined as “active physical activity” in the “Physical Activity Reference for Health Promotion 2013” in Japan. Practicing karate, especially sparring techniques, may help to enhance bone mineral density. It appears that longer duration, higher frequency, and earlier start of physical training positively influenced skeletal status.  Article visualizations

Nazım Hikmet'in vatan hainliği

Taha Toros Arşivi, Dosya Adı: Nazım Hikmet. Not: Gazetenin "Tersi Yüzü" köşesinde yayımlanmıştır.İstanbul Kalkınma Ajansı (TR10/14/YEN/0033) İstanbul Development Agency (TR10/14/YEN/0033

A Genome-Wide Association Analysis Identified a Novel Susceptible Locus for Pathological Myopia at 11q24.1

Myopia is one of the most common ocular disorders worldwide. Pathological myopia, also called high myopia, comprises 1% to 5% of the general population and is one of the leading causes of legal blindness in developed countries. To identify genetic determinants associated with pathological myopia in Japanese, we conducted a genome-wide association study, analyzing 411,777 SNPs with 830 cases and 1,911 general population controls in a two-stage design (297 cases and 934 controls in the first stage and 533 cases and 977 controls in the second stage). We selected 22 SNPs that showed P-values smaller than 10−4 in the first stage and tested them for association in the second stage. The meta-analysis combining the first and second stages identified an SNP, rs577948, at chromosome 11q24.1, which was associated with the disease (P = 2.22×10−7 and OR of 1.37 with 95% confidence interval: 1.21–1.54). Two genes, BLID and LOC399959, were identified within a 200-kb DNA encompassing rs577948. RT–PCR analysis demonstrated that both genes were expressed in human retinal tissue. Our results strongly suggest that the region at 11q24.1 is a novel susceptibility locus for pathological myopia in Japanese

Regulated C-C motif ligand 2 (CCL2) in luteal cells contributes to macrophage infiltration into the human corpus luteum during luteolysis

Intense macrophage infiltration is observed during luteolysis in various animals including women; however, we still do not know how macrophage infiltration into the human corpus luteum (CL) during luteolysis is regulated. In this study, we examined the expression, localization and regulation of an important chemokine for the recruitment of monocyte/macrophage lineages, C-C motif ligand 2 (CCL2), in the human CL across the luteal phase and in cultured human luteinized granulosa cells (LGCs), with special reference to the number of infiltrating macrophages and luteal cell function. CCL2 mRNA increased in the non-functional regressing CL during menstruation (P < 0.01), corresponding to an elevated mRNA expression of a macrophage-derived cytokine, tumor necrosis factor (TNF), and an increased number of infiltrating macrophages positively stained with a macrophage marker, CD68. CCL2 protein was immunohistochemically localized to the cytoplasm of granulosa-lutein and theca-lutein cells, and CCL2 mRNA was significantly reduced by hCG both in vivo (P < 0.05) and in vitro (P < 0.01). CCL2 was also down-regulated by luteotrophic prostaglandin (PG) E (P < 0.0001), but up-regulated by luteolytic PGF (P < 0.05) in vitro. Administration of TNF significantly enhanced the CCL2 mRNA expression in cultured LGCs (P < 0.01). A greater abundance of infiltrating macrophages were found around granulosa-lutein cells lacking 3 beta-HSD or PGE synthase (PGES) immunostaining. CCL2 mRNA expression was negatively correlated with both HSD3B1 and PGES, suggesting that locally produced progesterone and PGE suppress macrophage infiltration into the CL. Taken together, the infiltration of macrophages in the human CL is regulated by endocrine and paracrine molecules via regulation of the CCL2 expression in luteal cells.Supplementary data are available at http://molehr.oxfordjournals.org/http://molehr.oxfordjournals.org/lookup/suppl/doi:10.1093/molehr/gav028/-/DC

The Physics of the B Factories

This work is on the Physics of the B Factories. Part A of this book contains a brief description of the SLAC and KEK B Factories as well as their detectors, BaBar and Belle, and data taking related issues. Part B discusses tools and methods used by the experiments in order to obtain results. The results themselves can be found in Part C

A contribution of autopsies to understanding dementia in presenile-senile period(The 2nd Academic Meeting of Aomori University of Health and Welfare)

国立情報学研究所の「学術雑誌公開支援事業」により電子化されました