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  • 1
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    The Phenotypic and Genetic Spectrum of Paroxysmal Kinesigenic Dyskinesia in China
    Wiley ; 2020
    In:  Movement Disorders Vol. 35, No. 8 ( 2020-08), p. 1428-1437
    Details
    In: Movement Disorders, Wiley, Vol. 35, No. 8 ( 2020-08), p. 1428-1437
    Abstract: Paroxysmal kinesigenic dyskinesia is a spectrum of involuntary dyskinetic disorders with high clinical and genetic heterogeneity. Mutations in proline‐rich transmembrane protein 2 have been identified as the major pathogenic factor. Objectives We analyzed 600 paroxysmal kinesigenic dyskinesia patients nationwide who were identified by the China Paroxysmal Dyskinesia Collaborative Group to summarize the clinical phenotypes and genetic features of paroxysmal kinesigenic dyskinesia in China and to provide new thoughts on diagnosis and therapy. Methods The China Paroxysmal Dyskinesia Collaborative Group was composed of departments of neurology from 22 hospitals. Clinical manifestations and proline‐rich transmembrane protein 2 screening results were recorded using unified paroxysmal kinesigenic dyskinesia registration forms. Genotype‐phenotype correlation analyses were conducted in patients with and without proline‐rich transmembrane protein 2 mutations. High‐knee exercises were applied in partial patients as a new diagnostic test to induce attacks. Results Kinesigenic triggers, male predilection, dystonic attacks, aura, complicated forms of paroxysmal kinesigenic dyskinesia, clustering in patients with family history, and dramatic responses to antiepileptic treatment were the prominent features in this multicenter study. Clinical analysis showed that proline‐rich transmembrane protein 2 mutation carriers were prone to present at a younger age and have longer attack duration, bilateral limb involvement, choreic attacks, a complicated form of paroxysmal kinesigenic dyskinesia, family history, and more forms of dyskinesia. The new high‐knee‐exercise test efficiently induced attacks and could assist in diagnosis. Conclusions We propose recommendations regarding diagnostic criteria for paroxysmal kinesigenic dyskinesia based on this large clinical study of paroxysmal kinesigenic dyskinesia. The findings offered some new insights into the diagnosis and treatment of paroxysmal kinesigenic dyskinesia and might help in building standardized paroxysmal kinesigenic dyskinesia clinical evaluations and therapies. © 2020 International Parkinson and Movement Disorder Society
    Type of Medium: Online Resource
    ISSN: 0885-3185 , 1531-8257
    URL: Issue
    URL: Article
    DOI: 10.1002/mds.v35.8
    DOI: 10.1002/mds.28061
    RVK:
    XA 10000
    Language: English
    Publisher: Wiley
    Publication Date: 2020
    detail.hit.zdb_id: 2041249-6
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  • 2
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    Secretion of T at‐dependent halolysin S pt A capable of autocatalytic activation and its relation to haloarchaeal growth
    Wiley ; 2015
    In:  Molecular Microbiology Vol. 96, No. 3 ( 2015-05), p. 548-565
    Details
    In: Molecular Microbiology, Wiley, Vol. 96, No. 3 ( 2015-05), p. 548-565
    Abstract: Halolysins are T at‐dependent extracellular subtilases of haloarchaea. Whether halolysins can be activated before transport across the cytoplasmic membrane in a folded state and how haloarchaea minimize the risk of intracellular activation of halolysins and proteolysis of cellular proteins are unknown. Here, we report that both the precursor and proform of halolysin S pt A from N atrinema sp. J 7‐2 mature autocatalytically, and the S pt A maturation proceeds less efficiently in the presence of KCl than N a C l. When produced in H aloferax volcanii , most S pt A molecules are secreted into the culture medium, but a small number of molecules can be activated intracellularly, affecting the cell's growth. Furthermore, retardation of S pt A secretion in H fx. volcanii via mutation of the T at signal peptide leads to intracellular accumulation of the active enzyme and subsequent cell death. Although the S ec signal peptide can mediate S pt A secretion in H fx. volcanii , the secreted protein undergoes proteolysis. In N atrinema sp. J 7‐2, S pt A is secreted primarily during stationary phase, and the intracellular accumulation of mature enzyme occurs during the stationary and death phases. The growth phase‐dependent synthesis of S pt A , highly efficient secretion system, and high intracellular KC l concentration, contribute to the suppression of premature activation of this enzyme in N atrinema sp. J 7‐2.
    Type of Medium: Online Resource
    ISSN: 0950-382X , 1365-2958
    URL: Issue
    URL: Article
    DOI: 10.1111/mmi.2015.96.issue-3
    DOI: 10.1111/mmi.12955
    Language: English
    Publisher: Wiley
    Publication Date: 2015
    detail.hit.zdb_id: 1501537-3
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  • 3
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    Behavioral economics and monetary wisdom: A cross‐level analysis of monetary aspiration, pay (dis)satisfaction, risk perception, and corruption in 32 nations
    Wiley ; 2023
    In:  Business Ethics, the Environment & Responsibility Vol. 32, No. 3 ( 2023-07), p. 925-945
    Details
    In: Business Ethics, the Environment & Responsibility, Wiley, Vol. 32, No. 3 ( 2023-07), p. 925-945
    Abstract: Corruption involves greed, money, and risky decision‐making. We explore the love of money, pay satisfaction, probability of risk, and dishonesty across cultures. Avaricious monetary aspiration breeds unethicality. Prospect theory frames decisions in the gains‐losses domain and high‐low probability. Pay dissatisfaction (in the losses domain) incites dishonesty in the name of justice at the individual level. The Corruption Perceptions Index, CPI, signals a high‐low probability of getting caught for dishonesty at the country level. We theorize that decision‐makers adopt avaricious love‐of‐money aspiration as a lens and frame dishonesty in the gains‐losses domain (pay satisfaction‐dissatisfaction, Level 1) and high‐low probability (CPI, Level 2) to maximize expected utility and ultimate serenity. We challenge the myth: Pay satisfaction mitigates dishonesty across nations consistently. Based on 6500 managers in 32 countries, our cross‐level three‐dimensional visualization offers the following discoveries. Under high aspiration conditions, pay dissatisfaction excites the highest‐ (third‐highest) avaricious justice‐seeking dishonesty in high (medium) CPI nations, supporting the certainty effect. However, pay satisfaction provokes the second‐highest avaricious opportunity‐seizing dishonesty in low CPI entities, sustaining the possibility effect—maximizing expected utility. Under low aspiration conditions, high pay satisfaction consistently leads to low dishonesty, demonstrating risk aversion—achieving ultimate serenity. We expand prospect theory from a micro and individual‐level theory to a cross‐level theory of monetary wisdom across 32 nations. We enhance the S‐shaped Curve to three 3‐D corruption surfaces across three levels of the global economic pyramid, providing novel insights into behavioral economics, business ethics, the environment, and responsibility.
    Type of Medium: Online Resource
    ISSN: 2694-6416 , 2694-6424
    URL: Issue
    URL: Article
    DOI: 10.1111/beer.v32.3
    DOI: 10.1111/beer.12505
    Language: English
    Publisher: Wiley
    Publication Date: 2023
    detail.hit.zdb_id: 3059682-8
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  • 4
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    Characteristics of phenotypic antibiotic resistance of Helicobacter pylori and its correlation with genotypic antibiotic resistance: A retrospective study in Ningxia
    Wiley ; 2023
    In:  Helicobacter Vol. 28, No. 3 ( 2023-06)
    Details
    In: Helicobacter, Wiley, Vol. 28, No. 3 ( 2023-06)
    Abstract: Geographic differences exist in the antibiotic resistance patterns of Helicobacter pylori . Personalized treatment regimens based on local or individual resistance data are essential. We evaluated the current status of H. pylori resistance in Ningxia, analyzed resistance‐related factors, and assessed the concordance of phenotypic and genotypic resistance. Methods Strains were isolated from the gastric mucosa of patients infected with H. pylori in Ningxia and relevant clinical information was collected. Phenotypic antibiotic susceptibility assays (Kirby–Bauer disk diffusion) and antibiotic resistance gene detection (Sanger sequencing) were performed. Results We isolated 1955 H. pylori strains. The resistance rates of H. pylori to amoxicillin, levofloxacin, clarithromycin, and metronidazole were 0.9%, 42.4%, 40.4%, and 94.2%, respectively. Only five tetracycline‐resistant and one furazolidone‐resistant strain were identified. Overall, 3.3% of the strains were sensitive to all six antibiotics. Multidrug‐resistant strains accounted for 22.9%, of which less than 20% were from Wuzhong. Strains isolated from women and patients with nonulcerative disease had higher rates of resistance to levofloxacin and clarithromycin. Higher rates of resistance to metronidazole, levofloxacin, and clarithromycin were observed in the older age group than in the younger age group. The kappa coefficients of phenotypic resistance and genotypic resistance for levofloxacin and clarithromycin were 0.830 and 0.809, respectively, whereas the remaining antibiotics showed poor agreement. Conclusion H. pylori antibiotic resistance is severe in Ningxia. Therefore, furazolidone, amoxicillin, and tetracycline are better choices for the empirical therapy of H. pylori infection in this region. Host sex, age, and the presence of ulcerative diseases may affect antibiotic resistance of the bacteria. Personalized therapy based on genetic testing for levofloxacin and clarithromycin resistance may be a future direction for the eradication therapy of H. pylori infection in Ningxia.
    Type of Medium: Online Resource
    ISSN: 1083-4389 , 1523-5378
    URL: Issue
    URL: Article
    DOI: 10.1111/hel.v28.3
    DOI: 10.1111/hel.12960
    Language: English
    Publisher: Wiley
    Publication Date: 2023
    detail.hit.zdb_id: 2020336-6
    SSG: 12
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  • 5
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    Anatomical Variations of the Human Cochlea Determined from Micro‐CT and High‐Resolution CT Imaging and Reconstruction
    Wiley ; 2018
    In:  The Anatomical Record Vol. 301, No. 6 ( 2018-06), p. 1086-1095
    Details
    In: The Anatomical Record, Wiley, Vol. 301, No. 6 ( 2018-06), p. 1086-1095
    Abstract: Understanding the anatomical variations in the human cochlea is important for cochlear implants. This study examined these variations using a reconstructed fusion model of the skull and temporal bone. The three‐dimensional (3D) digital model of the temporal bone was reconstructed from multiple axial micro‐computed tomography (CT) scans of temporal bone and high resolution CT of the skull from 15 cadavers. A skull model was reconstructed and merged with the reconstructed temporal bone. The 3D relationship between the cochlea and the skull's mid‐sagittal plane was analyzed. The α and β angles of the cochlear autogenous rotation and bottom position, respectively, further subdivided the cochlear spatial orientation. The relationship between the base of the cochlea and the round window was evaluated with the Φ angle. Cochlear size was measured and the relationship was statistically analyzed. Cochlear implant electrode arrays were observed in five cases of right‐temporal bone specimens. The α, β, and Φ angles were 46.01 ± 9.65, 56.79 ± 3.58, and 44.41 ± 7.23, respectively. The α angle varied greatly and was negatively correlated to the Φ angle (correlation coefficient = −0.211, P   〈  0.05). Among the five specimens, the α and Φ angle of the 2R and 4R cochlear specimens was lower and higher than the mean value, respectively. These measurements revealed variations in the size and position of the cochlea. Some of these variations may require surgical adjustments for insertion of electrodes with cochlear implants and present a greater challenge for implantation of cochlear electrode implantation. These data also provide a better understanding of variations in human cochlear anatomy. Anat Rec, 301:1086–1095, 2018. © 2017 Wiley Periodicals, Inc.
    Type of Medium: Online Resource
    ISSN: 1932-8486 , 1932-8494
    URL: Issue
    URL: Article
    DOI: 10.1002/ar.v301.6
    DOI: 10.1002/ar.23730
    Language: English
    Publisher: Wiley
    Publication Date: 2018
    detail.hit.zdb_id: 2273240-8
    detail.hit.zdb_id: 2109216-3
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  • 6
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    TMEM151A Variants Cause Paroxysmal Kinesigenic Dyskinesia: A Large‐Sample Study
    Wiley ; 2022
    In:  Movement Disorders Vol. 37, No. 3 ( 2022-03), p. 545-552
    Details
    In: Movement Disorders, Wiley, Vol. 37, No. 3 ( 2022-03), p. 545-552
    Abstract: Paroxysmal kinesigenic dyskinesia (PKD) is the most common type of paroxysmal dyskinesias. Only one‐third of PKD patients are attributed to proline‐rich transmembrane protein 2 ( PRRT2 ) mutations. Objective We aimed to explore the potential causative gene for PKD. Methods A cohort of 196 PRRT2 ‐negative PKD probands were enrolled for whole‐exome sequencing (WES). Gene Ranking, Identification and Prediction Tool, a method of case–control analysis, was applied to identify the candidate genes. Another 325 PRRT2 ‐negative PKD probands were subsequently screened with Sanger sequencing. Results Transmembrane Protein 151 ( TMEM151A) variants were mainly clustered in PKD patients compared with the control groups. 24 heterozygous variants were detected in 25 of 521 probands (frequency = 4.80%), including 18 missense and 6 nonsense mutations. In 29 patients with TMEM151A variants, the ratio of male to female was 2.63:1 and the mean age of onset was 12.93 ± 3.15 years. Compared with PRRT2 mutation carriers, TMEM151A ‐related PKD were more common in sporadic PKD patients with pure phenotype. There was no significant difference in types of attack and treatment outcome between TMEM151A ‐positive and PRRT2 ‐positive groups. Conclusions We consolidated mutations in TMEM151A causing PKD with the aid of case–control analysis of a large‐scale WES data, which broadens the genotypic spectrum of PKD. TMEM151A ‐related PKD were more common in sporadic cases and tended to present as pure phenotype with a late onset. Extensive functional studies are needed to enhance our understanding of the pathogenesis of TMEM151A ‐related PKD. © 2021 International Parkinson and Movement Disorder Society
    Type of Medium: Online Resource
    ISSN: 0885-3185 , 1531-8257
    URL: Issue
    URL: Article
    DOI: 10.1002/mds.v37.3
    DOI: 10.1002/mds.28865
    RVK:
    XA 10000
    Language: English
    Publisher: Wiley
    Publication Date: 2022
    detail.hit.zdb_id: 2041249-6
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  • 7
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    Clinical guideline on magnetically controlled capsule gastroscopy (2021 edition)
    Wiley ; 2023
    In:  Journal of Digestive Diseases Vol. 24, No. 2 ( 2023-02), p. 70-84
    Details
    In: Journal of Digestive Diseases, Wiley, Vol. 24, No. 2 ( 2023-02), p. 70-84
    Abstract: With the development and generalization of endoscopic technology and screening, clinical application of magnetically controlled capsule gastroscopy (MCCG) has been increasing. In recent years, various types of MCCG are used globally. Therefore, establishing relevant guidelines on MCCG is of great significance. The current guidelines containing 23 statements were established based on clinical evidence and expert opinions, mainly focus on aspects including definition and diagnostic accuracy, application population, technical optimization, inspection process, and quality control of MCCG. The level of evidence and strength of recommendations were evaluated. The guidelines are expected to guide the standardized application and scientific innovation of MCCG for the reference of clinicians.
    Type of Medium: Online Resource
    ISSN: 1751-2972 , 1751-2980
    URL: Issue
    URL: Article
    DOI: 10.1111/cdd.v24.2
    DOI: 10.1111/1751-2980.13173
    Language: English
    Publisher: Wiley
    Publication Date: 2023
    detail.hit.zdb_id: 2317117-0
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  • 8
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    LRRC4 inhibits human glioblastoma cells proliferation, invasion, and proMMP‐2 activation by reducing SDF‐1α/CXCR4‐mediated ERK1/2 and Akt signaling pathways
    Wiley ; 2008
    In:  Journal of Cellular Biochemistry Vol. 103, No. 1 ( 2008-01), p. 245-255
    Details
    In: Journal of Cellular Biochemistry, Wiley, Vol. 103, No. 1 ( 2008-01), p. 245-255
    Abstract: Gliomas take a number of different genetic routes in the progression to glioblastoma multiforme, a highly invasive variant that is mostly unresponsive to current therapies. The α‐chemokine stromal cell‐derived factor (SDF)‐1α binds to the seven transmembrane G‐protein‐coupled CXCR‐4 receptor and acts to modulate cell migration and proliferation by activating multiple signal transduction pathways. Leucine‐rich repeats containing 4 (LRRC4), a putative glioma suppressive gene, inhibits glioblastoma cells tumorigenesis in vivo and cell proliferation and invasion in vitro. We also previously demonstrated that LRRC4 controlled glioblastoma cells proliferation by ERK/AKT/NF‐κB signaling pathway. In the present study, we demonstrate that CXC chemokine receptor 4 (CXCR4) is expressed in human glioblastoma U251 cell line, and that SDF‐1α increases the proliferation, chemotaxis, and invasion in CXCR4 + glioblastoma U251 cells through the activation of ERK1/2 and Akt. The reintroduction of LRRC4 in U251 cells inhibits the expression of CXCR4 and SDF‐1α/CXCR4 axis‐mediated downstream intracellular pathways such as ERK1/2 and Akt leading to proliferate, chemotactic and invasive effects. Furthermore, we provide evidence for proMMP‐2 activation involvement in the SDF‐1α/CXCR4 axis‐mediated signaling pathway. LRRC4 significantly inhibits proMMP‐2 activation by SDF‐1α/CXCR4 axis‐mediated ERK1/2 and Akt signaling pathway. Collectively, these results suggest a possible important “cross‐talk” between LRRC4 and SDF‐1α/CXCR4 axis‐mediated intracellular pathways that can link signals of cell proliferation, chemotaxis and invasion in glioblastoma, and may represent a new target for development of new therapeutic strategies in glioma. J. Cell. Biochem. 103: 245–255, 2008. © 2007 Wiley‐Liss, Inc.
    Type of Medium: Online Resource
    ISSN: 0730-2312 , 1097-4644
    URL: Issue
    URL: Article
    DOI: 10.1002/jcb.v103:1
    DOI: 10.1002/jcb.21400
    Language: English
    Publisher: Wiley
    Publication Date: 2008
    detail.hit.zdb_id: 1479976-5
    SSG: 12
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  • 9
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    Phenotypic analysis of atopic dermatitis in children aged 1–12 months: elaboration of novel diagnostic criteria for infants in China and estimation of prevalence
    Wiley ; 2019
    In:  Journal of the European Academy of Dermatology and Venereology Vol. 33, No. 8 ( 2019-08), p. 1569-1576
    Details
    In: Journal of the European Academy of Dermatology and Venereology, Wiley, Vol. 33, No. 8 ( 2019-08), p. 1569-1576
    Abstract: Atopic dermatitis ( AD ) is the most common skin disorder in infancy. However, the diagnosis and definite significance of infantile AD remains a debated issue. Objective To analyse the phenotypes of AD in infancy, to establish diagnostic criteria and to estimate the prevalence of this condition in China. Methods This is a multicentric study, in which 12 locations were chosen from different metropolitan areas of China. Following careful and complete history‐taking and skin examination, the definite diagnosis of AD was made and the severity based on the SCORAD index was determined by local experienced dermatologists. Based on the detailed phenotyping, the major and representative clinical features of infantile AD were selected to establish the diagnostic criteria and evaluate their diagnostic efficacy. Results A total of 5967 infants were included in this study. The overall point prevalence of AD was 30.48%. The infantile AD developed as early as at the second month of life, and its incidence peaked in the third month of life at 40.81%. The proportion of mild, moderate and severe AD was 67.40%, 30.57% and 2.03%, respectively. The most commonly seen manifestations in the infantile AD were facial dermatitis (72.07%), xerosis (42.72%) and scalp dermatitis (27.93%). We established the novel diagnostic criteria of infants, which included: (i) onset after 2 weeks of birth; (ii) pruritus and/or irritability and sleeplessness comparable with lesions; and (iii) all two items above with one of the following items can reach a diagnosis of AD : (i) eczematous lesions distributed on cheeks and/or scalp and/or extensor limbs, and (ii) eczematous lesions on any other parts of body accompanied by xerosis. Conclusions In China, the prevalence of AD in infancy is 30.48% according to clinical diagnosis of dermatologists. The novel Chinese diagnostic criteria for AD in infants show a higher sensitivity and comparable specificity.
    Type of Medium: Online Resource
    ISSN: 0926-9959 , 1468-3083
    URL: Issue
    URL: Article
    DOI: 10.1111/jdv.2019.33.issue-8
    DOI: 10.1111/jdv.15618
    Language: English
    Publisher: Wiley
    Publication Date: 2019
    detail.hit.zdb_id: 2022088-1
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  • 10
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    The efficacy and safety of fexuprazan in treating erosive esophagitis: a phase III, randomized, double‐blind, multicenter study
    Wiley ; 2024
    In:  Journal of Gastroenterology and Hepatology Vol. 39, No. 4 ( 2024-04), p. 658-666
    Details
    In: Journal of Gastroenterology and Hepatology, Wiley, Vol. 39, No. 4 ( 2024-04), p. 658-666
    Abstract: Fexuprazan is a novel potassium‐competitive acid blocker (P‐CAB). This study aimed to explore the noninferior efficacy and safety of fexuprazan to esomeprazole in treating erosive esophagitis (EE). Methods This was a phase III, randomized, double‐blind multicenter study. Patients with endoscopically confirmed EE were randomized to receive fexuprazan 40 mg or esomeprazole 40 mg once a daily for 4–8 weeks. The healing rates of EE, symptom response, GERD‐health‐related quality life (GERD‐HRQL), and treatment‐emergent adverse events (TEAEs) were compared between fexuprazan group and esomeprazole group. Results A total of 332 subjects were included in full analysis set (FAS) and 311 in per‐protocol set (PPS). The healing rates of fexuprazan and esomeprazole groups at 8 weeks were 88.5% (146/165) and 89.0% (145/163), respectively, in FAS and 97.3% (145/149) and 97.9% (143/146), respectively, in PPS. Noninferiority of fexuprazan compared with esomeprazole according to EE healing rates at 8 weeks was demonstrated in both FAS and PPS analysis. No significant difference was found between groups in EE healing rates at 4 weeks, symptom responses, and changes of GERD‐HRQL. The incidence of drug‐related AEs was 19.4% (32/165) in fexuprazan arm and 19.6% (32/163) in esomeprazole arm. Conclusion This study demonstrated noninferior efficacy of fexuprazan to esomeprazole in treating EE. The incidence of TEAEs was similar between fexuprazan and esomeprazole. Trial registration number NCT05813561.
    Type of Medium: Online Resource
    ISSN: 0815-9319 , 1440-1746
    URL: Issue
    URL: Article
    DOI: 10.1111/jgh.v39.4
    DOI: 10.1111/jgh.16471
    Language: English
    Publisher: Wiley
    Publication Date: 2024
    detail.hit.zdb_id: 2006782-3
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