Genetic and Clinical Profiles of Disseminated Bacillus Calmette-Guérin Disease and Chronic Granulomatous Disease in China

Background: Disseminated Bacillus Calmette-Guérin disease (D-BCG) in children with chronic granulomatous disease (CGD) can be fatal, while its clinical characteristics remain unclear because both diseases are extremely rare. The patients with CGD receive BCG vaccination, because BCG vaccination is usually performed within 24 h after delivery in China.Methods: We prospectively followed-up Chinese patients with CGD who developed D-BCG to characterize their clinical and genetic characteristics. The diagnoses were based on the patients' clinical, genetic, and microbiological characteristics.Results: Between September 2009 and September 2016, we identified 23 patients with CGD who developed D-BCG. Their overall 10-year survival rate was 34%. We created a simple dissemination score to evaluate the number of infected organ systems and the survival probabilities after 8 years were 62 and 17% among patients with simple dissemination scores of ≤3 and >3, respectively (p = 0.0424). Survival was not significantly associated with the CGD stimulation index or interferon-γ treatment. Eight patients underwent umbilical cord blood transplantation and 5 of them were successfully treated. The genetic analyses found mutations in CYBB (19 patients), CYBA (1 patient), NCF1 (1 patient), and NCF2 (1 patient). We identified 6 novel highly likely pathogenic mutations, including 4 mutations in CYBB and 2 mutations in NCF1.Conclusions: D-BCG is a deadly complication of CGD. The extent of BCG spreading is strongly associated with clinical outcomes, and hematopoietic stem cell transplantation may be a therapeutic option for this condition

Phenolic Composition from Different Loquat (Eriobotrya japonica Lindl.) Cultivars Grown in China and Their Antioxidant Properties

China is one of the most important centers of diversity for Eriobotrya japonica Lindl. in the world. In this study, seven loquat cultivars grown in China were evaluated for their phenolic compounds and antioxidant activity. Eleven phenolic compounds, i.e., 3-p-coumaroylquinincacid (3-p-CoQA), 5-caffeoylquinic acid (5-CQA), 4-caffeoylquinic acid (4-CQA), 3-caffeoylquinic acid (3-CQA), 5-feruloylquinic acid (5-FQA), quercetin-3-O-galactoside (Q-3-Gal), quercetin-3-O-glucoside (Q-3-Glu), quercetin-3-O-rhamnoside (Q-3-Rha), kaempferol-3-O-galactoside (K-3-Gal), kaempferol-3-O-rhamnoside (K-3-Rha), and kaempferol-3-O-glucoside (K-3-Glu) were identified and quantified in the peel and pulp of the cultivars tested. 3-CQA and 5-CQA were the predominant components in both fruit parts. 2,2-Diphenyl-1-picrylhydrazyl radicals (DPPH), 2,2'-azino-bis(3-ethylbenzothiazoline-6-sulfonate (ABTS), and ferric reducing antioxidant power (FRAP) assays were used for the antioxidant evaluation. Results showed that peel extracts had higher antioxidant activities than their pulp counterparts in all the cultivars tested, which was correlated with their higher total phenolic contents. The antioxidant potency composite (APC) index showed obvious variations ranging from 64.15 to 100 in the peel and from 59.49 to 97.95 in the pulp of different cultivars, where “Dahongpao” (DHP) and “Luoyangqing” (LYQ) had the highest APC index in the peel and pulp, respectively. Overall, loquat cultivars rich in hydroxycinnamic acids (HCAs) such as 3-p-CoQA, 5-CQA, 4-CQA, 3-CQA and 5-FQA showed relatively higher antioxidant activities, and may be excellent sources of phytochemicals and natural antioxidants

Nanoscale Assembly of Copper Bearing-Sleeve via Cold-Welding: A Molecular Dynamics Study

A bearing is an important component in contemporary machinery and equipment, whose main function is to support the mechanical rotator, reduce the friction coefficient during its movement, and guarantee the turning accuracy. However, assembly of a nanoscale bearing and sleeve is a challenging process for micro-nano mechanical manufacturers. Hence, we show the cold-welding mechanism of a copper nanobearing-nanosleeve via molecular dynamic simulations. We demonstrate that it is feasible to assemble a bearing and sleeve at the nanoscale to form a stable mechanism. The effect of temperature in the range of 150 to 750 K is investigated. As the temperature rises, the mechanical strength and the weld stress of the welded structures markedly decrease, accompanied by the observation of increasing disorder magnitude. This comparison study is believed to facilitate future mechanical processing and structural nano-assembly of metallic elements for better mechanical performance

Phenolic Composition and Antioxidant Properties of Different Peach [Prunus persica (L.) Batsch] Cultivars in China

China is an important centre of diversity for Prunus persica. In the present study, 17 Chinese peach cultivars were evaluated for phenolic content and antioxidant activity. Neochlorogenic acid (NCHA), chlorogenic acid (CHA), procyanidin B1 (B1), catechin (CAT), cyanidin-3-O-glucoside (C3G), quercetin-3-O-galactoside (Q3GAL), quercetin-3-O-glucoside (Q3GLU), quercetin-3-O-rutinoside (Q3R), and kaempferol-3-O-rutinoside (K3R) were identified and quantified. CHA and CAT were the predominant components in both the peel and pulp of this fruit. In general, peel extracts showed higher antioxidant activities than the pulp counterparts, consistent with the observed higher phenolic content. The melting peach cultivar “Xinyu” showed the highest antioxidant potency composite (APC) index. The principal component analysis (PCA) of peel phenolics showed a clear distinction between the melting peach and nectarine. Overall, peach cultivars rich in hydroxycinnamates and flavan-3-ols showed relatively higher antioxidant activities and might be excellent sources of phytochemicals and natural antioxidants

Cymbidium nipponicum Makino

原著和名: マヤラン科名: ラン科 = Orchidaceae採集地: 鹿児島県 種子島 生姜山 (大隅 種子島 生姜山)採集日: 1970/8/4採集者: 萩庭丈壽整理番号: JH015338国立科学博物館整理番号: TNS-VS-96533

Chemopreventive effect of flavonoids from Ougan (Citrus reticulata cv. Suavissima) fruit against cancer cell proliferation and migration

Citrus reticulata cv. Sucruissima (Ougan) is a rich source of flavonoids, and its peel has been employed as a traditional Chinese medicine for a long time. Among the different fruit parts, namely flayed, albedo, segment membrane and juice sacs, flavedo showed significant inhibitory effect on three cancer cell lines (HepG2, HL-60 and MDA-MB-231). Three flavanone glycosides, naringin, hesperidin, neohesperidin as well as four polymethoxylated flavones (PMFs), sinensetin, nobiletin, tangeretin and 5-demethylnobiletin were separated and purified from Ougan flavedo and identified by HPLC-DAD and LC-MS. Cytotoxicity assay showed that two flavanone aglycones and four PMFs exhibited significant antiproliferative activity on the three cancer cells. Structure-activity relationship was observed for the Ougan flavonoid-mediated antiproliferative activity. Wound healing assay showed that nobiletin and tangeretin exerted inhibitory effects on SKOV3 cell migration as well. These results indicate that Ougan flavonoids may have preventive or therapeutic potential for inhibiting cancer cell proliferation and migration. (C) 2014 Elsevier Ltd. All rights reserved

Quantitative N‑Glycomic and N‑Glycoproteomic Profiling of Peach [Prunus persica (L.) Batsch] during Fruit Ripening

Being part of the human diet, peach is an important fruit consumed worldwide. In the present study, a systematic first insight into the N-glycosylation of peach fruit during ripening was provided. First, N-glycome by reactive matrix-assisted laser desorption ionization time-of-flight (MALDI-TOF) mass spectrometry indicated that 6 of 24 N-glycans of peach were differentially expressed. Second, a comparative N-glycoproteome was characterized via18O-tagged N-glycosylation site labeling followed by nano-liquid chromatography-electrospray ionization-tandem mass spectrometry (nLC-ESI-MS/MS). Totally 1464 N-glycosites on 881 N-glycoproteins were identified, among which 291 N-glycosites on 237 N-glycoproteins were expressed differentially with a fold change value of 1.5 or 0.67. The enrichment analysis of GO and KEGG revealed that four pathways including other glycan degradation, phenylpropanoid biosynthesis, amino sugar and nucleotide sugar metabolism, and protein processing in endoplasmic reticulum were mainly enriched, in which several important N-glycoproteins with dynamic change during fruit ripening were further screened out. Our findings on a large scale for N-glycosylation analysis of peach fruit during ripening may provide new molecular insights for comprehending N-glycoprotein functions, which should be of great interest to both glycobiologists and analytical chemists