Search

Neuronal intranuclear inclusion disease (NIID) is a rare progressive neurodegenerative disorder, with complex and diverse of clinical manifestations characterized by eosinophilic hyaline inclusions in neurons and somatic cells. Due to the improvement in diagnostic methods, NIID is being increasingly diagnosed. Herein, we reported three NIID cases, which were diagnosed by skin biopsy and FMR1 gene, after DWI showed the characteristic corticomedullary junction hyperintensity. Then we reviewed all the published cases of NIID in PubMed, which were diagnosed by the same method. We discussed 15 NIID cases, including three cases diagnosed by us. The average age was 63.4 ± 14.0 years. The average time from onset of symptom to diagnosis was 5.4 ± 7.9 years. Nine cases had dementia or cognitive impairment. Three cases presented with encephalitis. Three cases showed bladder dysfunction and two cases only presented with dizziness and headache. Two cases showed acute neurological deficit mimicking stroke. All cases were diagnosed by skin biopsy, after DWI showed abnormal corticomedullary junction hyperintensity. Ten cases showed inclusions in sweat gland cells, and seven cases in adipocytes, sweat gland cells, and fibroblasts. EMG was performed in five cases, four of whom had abnormal results, showing simultaneous involvement of motor and sensory nerves. The results indicated that inclusions were more easily detected in sweat gland cells in skin biopsy. The early stage of NIID could only characterized by autonomic nerve function involvement. Combined autonomic nerve dysfunction might be another relatively common manifestation in NIID.

Let G = Hol(H) be the holomorph of a finite group H. If there is a prime q dividing ⋎H⋎ such that every q-central automorphism of H is inner and Z(H) = 1, then every Coleman automorphism of G is inner. In particular, the normalizer property holds for G.

A novel series of 1-substituted phenazines 4a-4l were designed and synthesized via Palladium-catalyzed reactions from 1-phenazine trifluoromethanesulfonate. These phenazines showed antichlamydial activity with IC50 values from 1 to 10 μM. Among them, compounds 4c and 4i exhibited the best antichlamydial activity with IC50 values from 2.06 to 2.74 μM without apparent cytotoxicity to host cells.

Neuronal intranuclear inclusion disease (NIID) is a rare progressive neurodegenerative disorder, with complex and diverse of clinical manifestations characterized by eosinophilic hyaline inclusions in neurons and somatic cells. Due to the improvement in diagnostic methods, NIID is being increasingly diagnosed.Herein, we reported three NIID cases, which were diagnosed by skin biopsy and FMR1 gene, after DWI showed the characteristic corticomedullary junction hyperintensity. Then we reviewed all the published cases of NIID in PubMed, which were diagnosed by the same method.We discussed 15 NIID cases, including three cases diagnosed by us. The average age was 63.4 ± 14.0 years. The average time from onset of symptom to diagnosis was 5.4 ± 7.9 years. Nine cases had dementia or cognitive impairment. Three cases presented with encephalitis. Three cases showed bladder dysfunction and two cases only presented with dizziness and headache. Two cases showed acute neurological deficit mimicking stroke. All cases were diagnosed by skin biopsy, after DWI showed abnormal corticomedullary junction hyperintensity. Ten cases showed inclusions in sweat gland cells, and seven cases in adipocytes, sweat gland cells, and fibroblasts. EMG was performed in five cases, four of whom had abnormal results, showing simultaneous involvement of motor and sensory nerves.The results indicated that inclusions were more easily detected in sweat gland cells in skin biopsy. The early stage of NIID could only characterized by autonomic nerve function involvement. Combined autonomic nerve dysfunction might be another relatively common manifestation in NIID.

Isolated cerebral mucormycosis is a clinical type of mucormycosis that is estimated to account for 8% of all mucormycosis cases. The clinical symptoms of isolated cerebral mucormycosis are elusive, and thus conventional techniques often lake sensitivity and specificity. Moreover, cultures are often negative, even when direct microscopy examination is positive. Although histopathology will probably remain the gold standard for the diagnosis of mucormycosis, obtaining a biopsy specimen is not always feasible in most vulnerable populations. Thus, molecular approaches are currently used as an advantageous assistant examination method to improve the early identification of the causative agent and subsequently guide therapy to improve the prognosis of patients. Here, we report a case of isolated cerebral mucormycosis caused by Rhizopus microspores in a healthy young adult that was identified using next-generation sequencing technology.

Cognitive impairment is a key clinical feature of ischemic leukoaraiosis (ILA); however, the underlying neurobiological mechanism is still unclear. ILA has been associated with widespread gray and white matter (WM) damage mainly located in cortical-cortical and cortico-subcortical pathways. A total of 36 patients with ILA (Fazekas rating score ≥2) and 31 healthy controls (HCs) underwent comprehensive neuropsychological assessments (covering four cognitive domains, i.e., information processing speed, episodic memory, executive and visuospatial function) and resting-state functional MRI scans. Graph theory-based analyses were employed to explore the topological organization of the brain connectome in ILA patients, and we further sought to explore the associations of connectome-based metrics and neuropsychological performances. An efficient small-world architecture in the functional brain connectome was observed in the ILA and control groups. Moreover, compared with the HCs, the ILA patients showed increased path length and decreased network efficiency (i.e., global and local efficiency) in their functional brain networks. Further network-based statistic (NBS) analysis revealed a functional-disconnected network in ILA, which is comprised of functional connections linking different brain modules (i.e., default mode, frontoparietal, ventral attention and limbic systems) and connections within single modules (i.e., ventral attention and limbic systems). Intriguingly, the abnormal network metrics correlated with cognitive deficits in ILA patients. Therefore, our findings provide further evidence to support the concept that ILA pathologies could disrupt brain connections, impairing network functioning, and cognition via a “disconnection syndrome.”

Background Constitutively activated nuclear factor kappa B (NF-κB) signalling plays vital roles in bladder urothelial carcinoma (BC) progression. We investigate the effect of receptor-interacting protein kinase 4 (RIPK4) on NF-κB activation and BC progression. Methods The expression of RIPK4 was examined in 25 cryopreserved paired bladder samples and 112 paraffin BC specimens. In vivo and in vitro assays were performed to validate effect of RIPK4 on NF-κB pathway-mediated BC progression. Results High expression of RIPK4 was observed in BC tissues and was an independent predictor for poor overall survival. Up or downregulating the expression of RIPK4 enhanced or inhibited, respectively, the migration and invasion of BC cells in vitro and in vivo. Mechanistically, RIPK4 promoted K63-linked polyubiquitination of tumour necrosis factor receptor-associated factor 2 (TRAF2), receptor-interacting protein (RIP) and NF-κB essential modulator (NEMO). RIPK4 also promoted nuclear localisation of NF-κB-p65, and maintained activation of NF-κB substantially, leading to upregulation of VEGF-A, ultimately promoting BC cell aggressiveness. Conclusions Our data highlighted the molecular aetiology and clinical significance of RIPK4 in BC: upregulation of RIPK4 contributes to NF-κB activation, and upregulates VEGF-A, and BC progression. Targeting RIPK4 might represent a new therapeutic strategy to improve survival for patients with BC.

Summary Objectives In the winter of 2013, people were facing the risk of human-to-human transmission of the re-emerging influenza A(H7N9) virus. We report herein information on the clinical features of two patients from the same family infected with this virus, the genomic sequences of the viruses harbored, and antiviral drug sensitivity. Methods Clinical and epidemiological data of two patients from the same family were collected and analyzed. Sequencing was done for the viruses isolated from these two patients and one epidemiologically related chicken, and the sequences of the eight gene segments of the viruses were analyzed phylogenetically. The sensitivity of the viruses to antiviral drug treatment was determined by neuraminidase inhibitor susceptibility test. Results The two patients from one family cluster shared the same symptoms but had different outcomes, and had a strong epidemiological link. Three strains, two from these two patients and one from the chicken, were isolated. Genome sequencing and analyses of phylogenetic trees demonstrated that the two viruses were almost identical. We noted the presence of the PB2 E627K amino acid substitution that was not present in isolates from the first wave, as well as two new mutations in the NA gene and six in the PB2 gene. Drug sensitivity testing showed that the new isolates were resistant to oseltamivir but sensitive to peramivir. Conclusions The two patients from one family cluster were probable human-to-human transmission cases. The new isolates were sensitive to peramivir but showed reduced sensitivity to oseltamivir.

Recent studies have revealed that not only fish but also rice consumption may significantly contribute to human exposure to mercury (Hg) in Asian countries. It is therefore essential to assess dietary exposure to Hg in rice and its associated health risk. However, risk assessments of Hg in rice in non-contaminated areas are generally lacking in Asian countries. In the present study, Hg concentrations were measured in rice samples collected from markets and supermarkets in Suzhou, a typical city in Eastern China. In addition, the rice ingestion rates (IR) were assessed via a questionnaire-based survey of Suzhou residents. The data were then used to assess the risk of Hg exposure associated with rice consumption, by calculating the hazard quotient (HQ). Hg contents in rice samples were well below the national standard (20 μg/kg), ranging from 1.46 to 8.48 ng/g. They were also significantly (p > 0.05) independent of the area of production and place of purchase (markets vs. supermarkets in the different districts). Our results indicate a low risk of Hg exposure from rice in Suzhou (HQ: 0.005–0.05), despite the generally high personal IR (0.05–0.4 kg/day). The risk of Hg associated with rice consumption for Suzhou residents was not significantly affected by the age or sex of the consumer (p > 0.05). Overall, our results provide a study of human exposure to Hg in rice in Chinese cities not known to be contaminated with Hg. Future studies should examine Hg exposure in different areas in China and in potentially vulnerable major food types.

The study aimed to compare the molecular mechanism of Porphuromonas gingivalis (P. gingivalis) and Aggregatibacter actinomycetemcomitans (A. actinomycetemcomitans). With microarray dataset (GSE9723) from Gene Expression Omnibus, differentially expressed genes (DEGs) were identified comparing normal cell samples with A. actinomycetemcomitans-infected and P. gingivalis-infected periodontitis cell samples, respectively (|logFC| > 1, p value

A majority of information in manufacturing is the process information which presents mobility. Such information reflects transforming process form materials to products. This research establishes a manufacturing process information model that presents uniqueness, validness, completeness and generality based in PSL. The model can provide necessary process information and related product, material and resource information required by systems for integrating and collaborative decision making. It then enables to build a collaborative manufacturing decision making process model which is capable of utilizing the process information model. By defining logic procedure, data wrapping and service invoking in the decision making process model, multi-manufacturing systems collaborative decision making will be implemented. Such method of manufacturing process information modeling and decision making process modeling is illustrated by examples of the collaborative creation of a part process plan and an assembly process plan considering actual manufacturing environment.

We selected 42 early-stage primary biliary cirrhosis (PBC) patients and 30 healthy controls (HC). Metagenomic sequencing of the 16S rRNA gene was used to characterize the fecal microbiome. UPLC-MS/MS assaying of small molecules was used to characterize the metabolomes of the serum, urine and feces. Liquid chip assaying of serum cytokines was used to characterize the immune profiles. The gut of PBC patients were depleted of some potentially beneficial bacteria, such as Acidobacteria, Lachnobacterium sp., Bacteroides eggerthii and Ruminococcus bromii, but were enriched in some bacterial taxa containing opportunistic pathogens, such as γ-Proteobacteria, Enterobacteriaceae, Neisseriaceae, Spirochaetaceae, Veillonella, Streptococcus, Klebsiella, Actinobacillus pleuropneumoniae, Anaeroglobus geminatus, Enterobacter asburiae, Haemophilus parainfluenzae, Megasphaera micronuciformis and Paraprevotella clara. Several altered gut bacterial taxa exhibited potential interactions with PBC through their associations with altered metabolism, immunity and liver function indicators, such as those of Klebsiella with IL-2A and Neisseriaceae with urinary indoleacrylate. Many gut bacteria, such as some members of Bacteroides, were altered in their associations with the immunity and metabolism of PBC patients, although their relative abundances were unchanged. Consequently, the gut microbiome is altered and may be critical for the onset or development of PBC by interacting with metabolism and immunity.

This research evaluated the planing performance of bamboo scrimber boards at three feed speeds and three cutting depths and used two methods to assess the finished surfaces. A macroscopic grade assessment was made according to ASTM D 1666-87 for visible planing defects. The other assessment was a microscopic scale roughness test measuring surface average roughness in terms of GB/T 12472. The visible results showed that the finished surface quality of bamboo resulted mainly in Grades and , with the main defect being torn grain. The two evaluations method showed consistent results in surface quality that the highest percentage of Grade was counted and the lowest roughness value occurred. Compared with the hardwood Sawtooth oak widely used in furniture industry in China, planning quality of bamboo scrimber board is better in terms of roughness.

In order to effectively represent assembly process information based on three dimensional (3D) models and rapidly extract the necessary information for assembly process planning, analysis and simulation, this paper aims to research the multiple view representation of assembly process information based on 3D models. Assembly process information based on 3D models is summarized and considered as the single source of the multiple view representation. The definition and organization of assembly process view is defined to implement assembly process representation based on view. Assembly process views based on assembly process planning work flow are established. View mapping approach is investigated to rapidly extract the necessary information for assembly process planning, analysis and simulation. The use of the multiple view assembly process representation is illustrated by building detailed assembly process view and assembly process simulation view and by extracting relevant information for the assembly process simulation view from detailed assembly process view.

The production organized in flexible manufacturing cells (FMC) can be a complicated issue when they are constrained by machines, robots, equipment and some other resources. Since machines and robots are the main bottleneck to the efficiency of FMC, this paper focused on the modeling and scheduling problem constrained by machines and robots. A common model representation, colored timed Petri net (CTPN) was utilized to build a FMC model constrained by robots and machines, which was then transformed to the simulation model. The scheduling problem was studied to establish a mathematical model of the FMC constrained by machines and robots. According to the model, a genetic algorithm was proposed to search an optimal solution by using an indirect coding of scheme. The effectiveness of the proposed algorithm was validated via an instance and the comparison with the result from the solution of simulated annealing algorithm.

Alperin and Broua have given the p-subpairs in a finite group, and proved that there is a Sylow theorem for p-subpairs. For a π-separable group with π-Hall subgroup nilpotent, we prove that there is a π-Sylow theorem for π-subpairs. Note that our π-subpairs are different from what Robinson and Staszewski gave.

Based on Paillier public key cryptosystem, a new threshold signature scheme is presented, which under RSA(N,N) assumption, is unforgeable and robust in random oracle model. The scheme enjoys the following properties: signature share gen- eration and verification are non-interactive; and the size of an in- dividual signature share is bounded by a constant times the size of the modulus N 2 .

Matrix-bound phosphine of anaerobic granular sludge was studied during methanogenic and acidogenic stage of anaerobic reactors. Results showed that at methanogenic stage, the concentration of matrix-bound phosphine of anaerobic sludge decreased as the height of reactor increased. But during acidogenic stage, matrix-bound phosphine disappeared when pH of the solution declined to 4-5. Further anaerobic static experiments showed that increased acidification and lower pH value promoted the decrease of matrix-bound phosphine. After cultivation with 2 000 mg x L(-1) glucose synthetic wastewater for two days, the matrix-bound phosphine fell from 1.76 ng x kg(-1) to 0.09 ng x kg(-1); At the same time, the highest disappearing rate of phosphine was reached 0.84 ng x (kg x d)(-1). While the glucose synthetic wastewater was 400 mg x L(-1), the disappearing rate of phosphine was only 0.27 ng x (kg x d)(-1). With further cultivation under acidogenic stage, the phosphine decreasing rate slowed down.

A practical system of B-ultrasonic is here introduced. By a special medical video card, the video image is digitized and captured dynamically or statically into computer. This system realizes a variety of functions such as the B-ultrasonic video image's acquisition and display, as well as the editing, processing, managing, storage, printing, It can build the database of patient's case history automatically. Together with other medical image workstation the system can be built as a PACS system of the hospital. And it can also act as an independent system.

AIM: To explore the possibility of the replacement of the gag gene between human immunodeficiency virus and bovine immunodeficiency virus, to achieve chimeric virions, and thereby gain a new kind of AIDS vaccine based on BHIV chimeric viruses. METHODS: A series of chimeric BHIV proviral DNAs differing in the replacement regions in gag gene were constructed, and then were transfected into 293T cells. The expression of chimeric viral genes was detected at the RNA and protein level. The supernatant of 293T cell was ultra centrifuged to detect the probable chimeric virion. Once the chimeric virion was detected, its biological activities were also assayed by infecting HIV-sensitive MT4 cells. RESULTS: Four chimeric BHIV proviral DNAs were constructed. Genes in chimeric viruses expressed correctly in transfected 293T cells. All four constructs assembled chimeric virions with different degrees of efficiency. These virions had complete structures common to retroviruses and packaged genomic RNAs, but the cleavages of the precursor Gag proteins were abnormal to some extent. Three of these virions tested could attach and enter into MT4 cells, and one of them could complete the course of reverse transcription. Yet none of them could replicate in MT4 cells. CONCLUSION: The replacement of partial gag gene of HIV with BIV gag gene is feasible. Genes in chimeric BHIVs are accurately expressed, and virions are assembled. These chimeric BHIVs (proviral DNA together with virus particles) have the potential to become a new kind of HIV/AIDS vaccine.