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Defective claudin-10 causes a novel variation of HELIX syndrome through compromised tight junction strand assembly

Genes & Diseases 2022年第5期9卷 1301-1314页

作者： Sebastian Sewerin jorg Piontek Ria Schonauer Sonja Grunewald Angelika Rauch Steffen Neuber Carsten Bergmann Dorothee Gunzel Jan halbritte Division of Nephrology University of Leipzig Medical CenterLeipzig 04103Germany Clinical Physiology/Nutritional Medicine Charitee Universitatsmedizin BerlinBerlin 12203Germany Division of Dermatology Venereologyand AllergologyUniversity of Leipzig Medical CenterLeipzig 04103Germany Division of Prosthodontics and Materials Science University of Leipzig Medical CenterLeipzig 04103Germany Center for Human Genetics BioscientiaIngelheim 55218Germany f Medizinische Genetik MainzLimbach GeneticsMainz 55128Germany

Formation of claudin-10 based tight junctions(TJs)is paramount to paracellular Na+transport in multiple *** variants in CLDN10 have been linked to HELIX syndrome,a salt-losing tubulopathy with altered handling of divalent cations accompanied by dysfunctional salivary,sweat,and lacrimal ***,we investigate molecular basis and phenotypic consequences of a newly identified homozygous CLDN10 variant that translates into a single amino acid substitution within the fourth transmembrane helix of *** addition to hypohidrosis(H),electrolyte(E)imbalance with impaired urine concentrating ability,and hypolacrimia(L),phenotypic findings include altered salivary electrolyte composition and amelogenesis imperfecta but neither ichthyosis(I)nor xerostomia(X).Employing cellular TJ reconstitution assays,we demonstrate perturbation of cis-and trans-interactions between mutant claudin-10 *** of reconstituted TJ strands show disturbed continuity and reduced abundance in the mutant ***,both major isoforms,claudin-10a and claudin-10b,are differentially affected with claudin-10b showing more severe molecular ***,expression of the mutant in renal epithelial cells with endogenous TJs results in wild-type-like ion selectivity and conductivity,indicating that aberrant claudin-10 is generally capable of forming functional paracellular ***,mutant proteins prove pathogenic by compromising claudin-10 TJ strand *** ex vivo investigations indicate their insertion into TJs to occur in a tissue-specific manner.

关键词： Claudin-10 HELIX syndrome Paracellular transport Salt-losing tubulopathy Tight junction

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A New β-Estradiol-lnducible Vector Set that Facilitates Easy Construction and Efficient Expression of Transgenes Reveals CBL3-Dependent Cytoplasm to Tonoplast Translocation of CIPK5

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Molecular Plant 2013年第6期6卷 1814-1829页

作者： Kathrin Schliicking Kai H. Edel Maria M. Drerupa Philipp Koster Christian Eckert Leonie Steinhorst Rainer Waadt Oliver Batistic jorg Kudla Institut for Biologie und Biotechnologie der Pflanzen Universit~t MQnster Schlossplatz 4 48149 MQnster Gel'many _ . _ Present address: University of California San Diego Division of Biological Sciences Cell and Developmental Biology Section 9500 Gilman Drive #0116 LaJolla CA 92093-0116 USA

Transient and stable expression of transgenes is central to many investigations in plant biology research. Chemical regulation of expression can circumvent problems of plant lethality caused by constitutive overexpression or allow inducible knock （out/down） approaches. Several chemically inducible or repressible systems have been described and successfully applied. However, cloning and application-specific modification of most available inducible expression systems have been limited and remained complicated due to restricted cloning options. Here we describe a new set of 57 vectors that enable transgene expression in transiently or stably transformed cells. All vectors harbor a synthetically optimized XVE expression cassette, allowing I^-estradiol mediated protein expression. Plasmids are equipped with the reporter genes GUS, GFP, mCherry, or with ha and Strepll epitope tags and harbor an optimized multiple cloning site for flexible and simple clon- ing strategies. Moreover, the vector design allows simple substitution of the driving promoter to achieve tissue-specificity or to modulate expression ranges of inducible transgene expression. We report details of the kinetics and dose-dependence of expression induction in Arabidopsis leaf mesophyll protoplasts, transiently transformed Nicotiana benthamiana leaves, and stably transformed Arabidopsis plants. Using these vectors, we investigated the influence of CBL （Calcineurin B-like） protein expression on the subcellular localization of CIPKs （Calcineurin B-like interacting protein kinases）. These analyses uncovered that induced co-expression of CBL3 is fully sufficient for dynamic translocation of CIPK5 from the cytoplasm to the tonoplast. Thus, the vector system presented here facilitates a broad range of research applications.

关键词： vector inducible gene expression βestradiol Arabidopsis CBL CIPK.

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Temperament and character as predictor of health related quality of life after metacarpophalangeal joint arthroplasty ——Personality and MCP joint arthroplasty outcome

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Health 2010年第9期2卷 1101-1109页

作者： Sven Brandstrom Kurt Pettersson jorg Richter Department of Neuroscience and Locomotion Division of Psychiatry Linkoping UniversityLinkopingSweden Department of hand Surgery Orebro University HospitalOrebroSweden Department of Psychology Center for Child and Adolescent Mental HealthOsloNorway

Purpose: To evaluate personality characteris- tics’ impact upon outcome after silicone-based MP arthroplasty in RA patients. Methods: 40 RA patients who had undergone operations on their MP joints were investigated in a one-year fol- low-up. Objective measurement to assess grip strength and active range of motion—Paper- pencil-tests to assess pain during activity and at rest performance, QoL, and personality. Results: Significant improvement was observed in func- tion and pain related scores except for the pain related VAS and in several QoL facets and do- mains. Patients who experienced improvement reported higher scores on the activities of daily living facet of the WHO QoL questionnaire. Those with lower pain showed more independ- ence. The variance of the QoL domain scores, other than social and physical domains, could substantially and meaningfully be explained by variance of objective measures combined with personality scores. Conclusions: Most RA pa- tients’ QoL can be improved by MP arthroplasty despite remaining substantial level of pain. NS and ha seem to play an important role in the adaptation process during the long term, chronic illness;whereas SD represents a tool of coping with the burden of pain and disability. Personal- ity characteristics are highly predictive for QoL suggesting their important mediating role be- tween experienced pain and disability and HR QoL.

关键词： Rheumatoid Arthritis Health Related Quality of Life MP Joint Arthroplasty Temperament Character

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Molecular Plant 2012年第4期5卷 951-954页

作者： Giuseppe Ortu Raffaella Balestrini Patricia A. Pereira jorg D. Becker Helge Kuste Paola Bonfante Department of Life Science and Systems Biology University of Torino Italy Istituto per la Protezione delle Piante--CNR Torino Italy Instituto Gulbenkian de Ciencia 2780-156 Oeiras Portugal Institut for Pflanzengenetik Leibniz Universitat Hannover Herrenhauser Str. 2 D-30419 Hannover Germany

Dear Editor, Phytohormones are essential regulators of plant develop- ment, but their role in the signaling processes between plants and fungi during arbuscular mycorrhizal （AM） establishment is far from being understood （Ludwig-M011er, 2010）. AM coloni- zation leads to extensive effects on host metabolism, as revealed by transcriptome studies of AM plants （Hogekamp et al., 2011）. Some genes have been specified as an AM core set, since they are mycorrhizal-responsive, irrespective of the identity of the plant,

关键词： AM真菌植物激素相关基因早期阶段调节过程生物合成相互作用赤霉素

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Instantly Investigating the Adsorption of Polymeric Corrosion Inhibitors on Magnesium Alloys by Surface Analysis under Ambient Conditions

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Journal of Surface Engineered Materials and Advanced Technology 2014年第5期4卷 282-294页

作者： Livia M.Garcia Goncalves Larissa C.Sanchez Stephani Stamboroski Yendry R.Corrales Urena Welchy Leite Cavalcanti jorg Ihde Michael Noeske Marko Soltau Kai Brune Fraunhofer Institute for Manufacturing Technology and Advanced Materials IFAM BremenGermany CAPES Foundation Ministry of Education of BrazilBrasiliaBrazil Department of Materials Engineering UFSCarFederal University of Sao CarlosSao CarlosBrazil Department of Chemistry UFSCFederal University of Santa CatarinaFlorianopolisBrazil UNESP Sao Paulo State University Sao PauloBrazil Straetmans High TAC GmbH HamburgGermany

Surface engineering of magnesium alloys requires adequate strategies, processes and materials permitting corrosion protection. Liquid formulations containing corrosion inhibitors often are to be optimized according to the demands of the respective substrate and following the service conditions during its application. As an interdisciplinary approach, a combination of several techniques for instantly monitoring or elaborately analyzing the surface state of magnesium was accomplished in order to characterize the performance of new adsorbing sustainable amphiphilic polymers which recently were developed to facilitate a multi-metal corrosion protection approach. The application of established techniques like Contact Angle measurements and X-ray Photoelectron Spectroscopy investigations was supplemented by introducing related and yet faster online-capable and larger-scale techniques like Aerosol Wetting Test and Optically Stimulated Electron Emission. Moreover, an inexpensive setup was configured for scaling the inset and the extent of degradation processes which occur at local electrochemical circuits and lead to hydrogen bubble formation. Using these analytical tools, changes of the surface state of emeried AM50 samples were investigated. Even in contact with water, being a moderate corrosive medium, the online techniques facilitated detecting surface degradation of the unprotected magnesium alloy within some seconds. In contrast, following contact with a 1 weight% formulation of a polymeric corrosion inhibitor, surface monitoring indicated a delay of the onset of degradation processes by approximately two orders of magnitude in time. Mainly based on the spectroscopic investigations, the corrosion inhibiting effects of the investigated polymer are attributed to the adsorption of a primary polymer layer with a thickness of a few nanometers which occurs within some seconds. Immersion of magnesium for several hours brings up a protective film with around ten nanometers th

关键词： Online Surface Monitoring Magnesium Alloys Polymeric Corrosion Inhibitors Fast Screening of Effective Formulations Optimization of Application Process

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