Complex phenotype in Fanconi renotubular syndrome type 1: Hypophosphatemic rickets as the predominant presentation.

GATM-related Fanconi renotubular syndrome 1 (FRTS1) is a form of renal Fanconi syndrome (RFS), which is a disorder of solute and water reabsorption caused by defects in the function of the entire proximal tubule. Recent findings reveal the molecular basis of FRTS1: Intramitochondrial fiber aggregation triggered by mutant GATM provides a starting point for proximal tubule damage and drives disease progression. As a rare and newly recognized inherited kidney disease, the complex manifestations of FRTS1 are easily underdiagnosed or misdiagnosed.

RNA-seq reveals tight junction-relevant erythropoietic fate induced by OCT4 in human hair follicle mesenchymal stem cells.

Background: Human hair follicle mesenchymal stem cells (hHFMSCs) isolated from hair follicles possess multilineage differentiation potential. OCT4 is a gene critically associated with pluripotency properties. The cell morphology and adhesion of hHFMSCs significantly changed after transduction of OCT4 and two subpopulations emerged, including adherent cells and floating cell.

Adsorption mechanism of rhein-coated FeO as magnetic adsorbent based on low-field NMR.

In the present study, a magnetic adsorbent, rhein-coated magnetic FeO nanoparticle (RMNP), for Pb and Mg had been developed, and adsorption mechanism was studied via low-field NMR. RMNP was characterized by TEM, FTIR, and XRD. RMNP could adsorb and remove Pb and Mg from water and was successfully applied to remove Pb and Mg from wastewater, with satisfactory recovery rates and high adsorption capacities.

Gastric Mucosa-Associated Microbial Signatures of Early Gastric Cancer.

Alterations in the microbiome are associated with the development of gastric cancer. Our study aimed to identify dysbiotic features in early gastric cancer (EC). The gastric microbiome was assessed in EC ( = 30), advanced gastric cancer (AC) ( = 30), and chronic gastritis (CG) ( = 60).

A Novel Anti-Microbial Peptide from Pseudomonas, REDLK Induced Growth Inhibition of Leishmania tarentolae Promastigote In Vitro.

Leishmaniasis is a prevalent cause of death and animal morbidity in underdeveloped countries of endemic area. However, there is few vaccine and effective drugs. Antimicrobial peptides are involved in the innate immune response in many organisms and are being developed as novel drugs against parasitic infections.

In vitro leishmanicidal activity of antimicrobial peptide KDEL against Leishmania tarentolae.

Leishmaniasis, caused by the intracellular protozoan parasite Leishmania, remains an important neglected tropical infectious disease. Infection may be lethal if untreated. Currently, the available drugs for the disease are limited by high toxicity and drug resistance.

Metabolism of hyperpolarized C-acetoacetate to β-hydroxybutyrate detects real-time mitochondrial redox state and dysfunction in heart tissue.

Mitochondrial dysfunction is considered to be an important component of many metabolic diseases yet there is no reliable imaging biomarker for monitoring mitochondrial damage in vivo. A large prior literature on inter-conversion of β-hydroxybutyrate and acetoacetate indicates that the process is mitochondrial and that the ratio reflects a specifically mitochondrial redox state. Therefore, the conversion of [1,3- C]acetoacetate to [1,3- C]β-hydroxybutyrate is expected to be sensitive to the abnormal redox state present in dysfunctional mitochondria.

Trichomonas vaginalis triggers the release of THP-1 extracellular traps.

Trichomonas vaginalis is responsible for the prevalence of trichomoniasis, which may be one of the most epidemic nonviral sexually transmitted pathogens. Extracellular traps (ET) are a unique form of innate immunity against infection; they bind to and kill microorganisms. However, the effect of T.

Sensitive fluorescent assay for copper(ii) determination in aqueous solution using quercetin-cyclodextrin inclusion.

Environmentally friendly probe materials for detecting copper ions were studied in this research. Fluorescent emission of quercetin (Q) was observed in the buffer solution (pH = 7.40), and (2-hydroxypropyl)-β-cyclodextrin (CD) could enhance the fluorescence intensity of Q.

Esterase-Catalyzed Production of Hyperpolarized C-Enriched Carbon Dioxide in Tissues for Measuring pH.

C Magnetic resonance imaging of hyperpolarized (HP) C-enriched bicarbonate (HCO) and carbon dioxide (CO) is a novel and sensitive technique for tissue pH mapping in vivo. Administration of the HP physiological buffer pair is attractive, but poor polarization and the short T of C-enriched inorganic bicarbonate salts are major drawbacks for this approach. Here, we report a new class of mixed anhydrides for esterase-catalyzed production of highly polarized CO and HCO in tissue.

A new malyngamide from the marine cyanobacterium Moorea producens.

A new malyngamide (1) was isolated along with seven known compounds (2-8) from the marine cyanobacterium Moorea producens collected in Hawaii. Compound 1 represented the first reported malyngamide with a hydroxy moiety at C-7 of the characteristic fatty acid portion of the compound. Compound 1 showed cytotoxicity against L1210 cell line at an IC value of 2.

[A Quercetin-Based Fluorescent Probe for the Recognition of Fluorid Ions].

Quercetin (Q) is one of the most common flavonoids present in roots, stems, leaves, flowers and fruits of most plants. In this study, a quercetin-based fluorescent probe for detecting fluorid ions had been proposed. With good selectivity and sensitivity for fluorid ions, Q-based fluorescent probe was easier to prepare, more eco-friendly and more innoxious compared with traditional fluorescent probe obtained by organic chemistry synthesis operation.

A new diatom growth inhibition assay using the XTT colorimetric method.

Marine biofouling, which leads to significant operational stress and economic damage on marine infrastructures, is a major problem in marine related industries. Currently, the most common way to avoid marine biofouling involves the use of biocidal products in surface coatings. However, the need for environmentally friendly antibiofouling compounds has increased rapidly with the recent global prohibition of harmful antifoulants, such as tributyltin (TBT).

Hyperpolarized 15N-pyridine derivatives as pH-sensitive MRI agents.

Highly sensitive MR imaging agents that can accurately and rapidly monitor changes in pH would have diagnostic and prognostic value for many diseases. Here, we report an investigation of hyperpolarized (15)N-pyridine derivatives as ultrasensitive pH-sensitive imaging probes. These molecules are easily polarized to high levels using standard dynamic nuclear polarization (DNP) techniques and their (15)N chemical shifts were found to be highly sensitive to pH.

Two new lyngbyatoxin derivatives from the Cyanobacterium, Moorea producens.

The toxin-producing cyanobacterium, Moorea producens, is a known causative organism of food poisoning and seaweed dermatitis (also known as "swimmer's itch"). Two new toxic compounds were isolated and structurally elucidated from an ethyl acetate extract of M. producens collected from Hawaii.

Silencing RhoA inhibits migration and invasion through Wnt/β-catenin pathway and growth through cell cycle regulation in human tongue cancer.

Ras homolog gene family member A (RhoA) has been identified as a critical regulator of tumor aggressive behavior. In this study, we assessed the role of RhoA in the mechanisms underlying growth, migration, and invasion of squamous cell carcinoma of tongue (TSCC). Stable RhoA knockdown of TSCC cell lines SCC-4 and CAL27 were achieved using Lentiviral transfection.

Colon cancer bears overexpression of OTUB1.

Objective: OTUB1 is a member of deubiquitinating enzymes; however, its expression and function in colon cancer are still unclear. The present study aimed at investigating the expression of OTUB1 in colon cancer and the relationship between the expression and some clinicopathologic parameters.

Methods: Immunohistochemistry and quantitative real-time PCR were carried out in selected colon cancer and normal mucosa tissues.

A new lyngbyatoxin from the Hawaiian cyanobacterium Moorea producens.

Lyngbyatoxin A from the marine cyanobacterium Moorea producens (formerly Lyngbya majuscula) is known as the causative agent of "swimmer's itch" with its highly inflammatory effect. A new toxic compound was isolated along with lyngbyatoxin A from an ethyl acetate extract of M. producens collected from Hawaii.

[Primary drug resistance of HIV-1 infected men who have sex with men in Shenzhen, China].

Objective: To evaluate the status of drug resistance among treat-naive HIV-1 infected men who have sex with men (MSM) in Shenzhen during the period of 2008 - 2010.

Methods: Plasma samples of 227 treatment-naive HIV-1 infected MSM were collected in Shenzhen. HIV-1 pol genes (RT and PR) were amplified by nested-polymerase chain reaction (PCR) from RNA.

Photoinduced hydrogen evolution in supramolecular devices with a rhenium photosensitizer linked to FeFe-hydrogenase model complexes.

Coordination of the pyridyl-attached diiron azadithiolate hexacarbonyl complexes (2 and 3) through the pyridyl nitrogen to the Re on 10-phenanthroline rhenium (5a) and 2,9-diphenyl-1,10-phenanthroline rhenium (5b) forms novel [Re-Fe] complexes 7a, 7b and 8 respectively. Under visible light illumination using triethylamine as a sacrificial electron donor and [Re-Fe] type complexes (7a, 7b or 8) as catalysts, remarkably increased efficiency was observed for photoinduced hydrogen production with a turnover number reaching 11.8 from complex 7a and 8.

OTUB1 overexpression in mesangial cells is a novel regulator in the pathogenesis of glomerulonephritis through the decrease of DCN level.

Background: OTUB1 is a member of OTUs (Ovarian-tumor-domain-containing proteases), a deubiquitinating enzymes family (DUBs), which was shown as a proteasome-associated DUB to be involved in the proteins Ub-dependent degradation. It has been reported that OTUB1 was expressed in kidney tissue. But its concrete cellular location and function in the kidney remain unclear.

Role of cross-talk between the Smad2 and MAPK pathways in TGF-beta1-induced collagen IV expression in mesangial cells.

Transforming growth factor beta1 (TGF-beta1) can promote sclerosis in many kidney diseases by enhancing the synthesis of collagens. However, the mechanisms of down-stream intracellular signal transduction in TGF-beta1-induced collagen synthesis is not fully understood. The purpose of this study was to further investigate the mechanisms and the cross-talk between the MAPK and Smad2 pathways.