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排序方式: 共有339条查询结果,搜索用时 62 毫秒
1.
Daniel J. Owens Julien Messant Sophie Moog Mark Viggars Arnaud Ferry Kamel Mamchaoui Emmanuelle Lacne Norma Romro Astrid Brull Gisle Bonne Gillian Butler-Browne Catherine Coirault 《International journal of molecular sciences》2021,22(1)
Laminopathies are a clinically heterogeneous group of disorders caused by mutations in the LMNA gene, which encodes the nuclear envelope proteins lamins A and C. The most frequent diseases associated with LMNA mutations are characterized by skeletal and cardiac involvement, and include autosomal dominant Emery–Dreifuss muscular dystrophy (EDMD), limb-girdle muscular dystrophy type 1B, and LMNA-related congenital muscular dystrophy (LMNA-CMD). Although the exact pathophysiological mechanisms responsible for LMNA-CMD are not yet understood, severe contracture and muscle atrophy suggest that mutations may impair skeletal muscle growth. Using human muscle stem cells (MuSCs) carrying LMNA-CMD mutations, we observe impaired myogenic fusion with disorganized cadherin/β catenin adhesion complexes. We show that skeletal muscle from Lmna-CMD mice is unable to hypertrophy in response to functional overload, due to defective fusion of activated MuSCs, defective protein synthesis and defective remodeling of the neuromuscular junction. Moreover, stretched myotubes and overloaded muscle fibers with LMNA-CMD mutations display aberrant mechanical regulation of the yes-associated protein (YAP). We also observe defects in MuSC activation and YAP signaling in muscle biopsies from LMNA-CMD patients. These phenotypes are not recapitulated in closely related but less severe EDMD models. In conclusion, combining studies in vitro, in vivo, and patient samples, we find that LMNA-CMD mutations interfere with mechanosignaling pathways in skeletal muscle, implicating A-type lamins in the regulation of skeletal muscle growth. 相似文献
2.
Thomas C. van den Ende Jeroen M. M. Heuts Dr. Geoffroy P. P. Gential Marten Visser Michel J. van de Graaff Dr. Nataschja I. Ho Prof. Dr. Wim Jiskoot Dr. A. Rob P. M. Valentijn Nico J. Meeuwenoord Prof. Dr. Herman S. Overkleeft Prof. Dr. Jeroen D. C. Codée Prof. Dr. Sjoerd H. van der Burg Dr. Els M. E. Verdegaal Prof. Dr. Gijsbert A. van der Marel Prof. Dr. Ferry Ossendorp Dr. Dmitri V. Filippov 《Chembiochem : a European journal of chemical biology》2021,22(7):1215-1222
Synthetic vaccines, based on antigenic peptides that comprise MHC−I and MHC-II T-cell epitopes expressed by tumors, show great promise for the immunotherapy of cancer. For optimal immunogenicity, the synthetic peptides (SPs) should be adjuvanted with suitable immunostimulatory additives. Previously, we have shown that improved immunogenicity in vivo is obtained with vaccine modalities in which an SP is covalently connected to an adjuvanting moiety, typically a ligand to Toll-like receptor 2 (TLR2). SPs were covalently attached to UPam, which is a derivative of the classic TLR2 ligand Pam3CysSK4. A disadvantage of the triply palmitoylated UPam is its high lipophilicity, which precludes universal adoption of this adjuvant for covalent modification of various antigenic peptides as it renders the synthetic vaccine insoluble in several cases. Here, we report a novel conjugatable TLR2 ligand, mini-UPam, which contains only one palmitoyl chain, rather than three, and therefore has less impact on the solubility and other physicochemical properties of a synthetic peptide. In this study, we used SPs that contain the clinically relevant neoepitopes identified in a melanoma patient who completely recovered after T-cell therapy. Homogeneous mini-UPam-SP conjugates have been prepared in good yields by stepwise solid-phase synthesis that employed a mini-UPam building block pre-prepared in solution and the standard set of Fmoc-amino acids. The immunogenicity of the novel mini-UPam-SP conjugates was demonstrated by using the cancer patient's T-cells. 相似文献
3.
Cationic Nanogels: Reduction‐Sensitive Dextran Nanogels Aimed for Intracellular Delivery of Antigens (Adv. Funct. Mater. 20/2015) 下载免费PDF全文
4.
The fire behavior of various large samples polymers assemblies is an under‐researched topic. In fire risk assessment, the resultant heat release rate of burning different combustibles has to be known. To highlight interactions between components, 2 types of configurations were tested: juxtaposed and layered materials, using a specific radiant panel setup. For juxtaposed assemblies, results indicated that the more flammable component acted as an accelerator for the global combustion kinetics. For layered assemblies, 2 main phenomena were evidenced: the front material acted as a shield delaying the combustion of the backside material and the presence of a backside material induced a thermal thickening that slowed down the combustion of the front material. The experimental burning behaviors of the assembly were compared with a simulated one calculated from the superposition principle. This method was described by introducing a time offset and/or a slowdown factor in the model, confirmed with the use of different assemblies. 相似文献
5.
Batch tests were performed to investigate chemical and physical processes that may result during electrolytic aeration of a contaminated aquifer using naphthalene as a model contaminant. Naphthalene degradation of 58-66% took place electrolytically and occurred at the same rates at a pH of 4 and 7. 1,4-naphthoquinone was identified as a product of the electrolysis. Stripping due to gases produced at the electrodes did not result in any naphthalene loss. Hydrogen peroxide (which may be produced at the cathode) did not have any effect on naphthalene, but the addition of ferrous iron (which may be present in aquifers) resulted in 67-99% disappearance of naphthalene. Chlorine (which may be produced from the anodic oxidation of chloride) can effectively degrade naphthalene at pH of 4, but not at a pH of 7. Mono-, di- and poly chloronaphthalenes were identified as oxidation products. Ferric iron coagulation (due to the oxidation of ferrous iron) did not significantly contribute to naphthalene loss. Overall, electrolytic oxidation and chemical oxidation due to the electrolytic by-products formed are significant abiotic processes that could occur and should be accounted for if bioremediation of PAH-contaminated sites via electrolytic aeration is considered. Possible undesirable products such as chlorinated compounds may be formed when significant amounts of chlorides are present. 相似文献
6.
Grundwasser - A significant proportion of the global water supply is ensured by karst aquifers. However, these are often highly vulnerable to contamination. A storm water tank... 相似文献
7.
Meinier Romain Fellah Mahdi Sonnier Rodolphe Zavaleta Pascal Suard Sylvain Ferry Laurent 《Fire Technology》2022,58(2):689-707
Fire Technology - The ignition of four different PVC-based electric cables was studied using cone calorimeter and the influence of the charring phenomenon on ignition was investigated. The... 相似文献
8.
Dandan Li Neda Kordalivand Marieke F. Fransen Ferry Ossendorp Koen Raemdonck Tina Vermonden Wim E. Hennink Cornelus F. van Nostrum 《Advanced functional materials》2015,25(20):2993-3003
Targeting of antigens to dendritic cells (DCs) to induce strong cellular immune response can be established by loading in a nano‐sized carrier and keeping the antigen associated with the particles until they are internalized by DCs. In the present study, a model antigen (ovalbumin, OVA) is immobilized in cationic dextran nanogels via disulfide bonds. These bonds are stable in the extracellular environment but are reduced in the cytosol of DCs due to the presence of glutathione. Reversible immobilization of OVA in the nanogels is demonstrated by the fact that hardly any release of the protein occurred at pH 7 in the absence of glutathione, whereas rapid release of OVA occurs once the nanogels are incubated in buffer with glutathione. Furthermore, these OVA conjugated nanogels show intracellular release of the antigen in DCs and boost the MHC class I antigen presentation, demonstrating the feasibility of this concept for the aimed intracellular antigen delivery. 相似文献
9.
10.
Vivian E. Ferry Jeremy N. Munday Harry A. Atwater 《Advanced materials (Deerfield Beach, Fla.)》2010,22(43):4794-4808
This paper reviews the recent research progress in the incorporation of plasmonic nanostructures with photovoltaic devices and the potential for surface plasmon enhanced absorption. We first outline a variety of cell architectures incorporating metal nanostructures. We then review the experimental fabrication methods and measurements to date, as well as systematic theoretical studies of the optimal nanostructure shapes. Finally we discuss photovoltaic absorber materials that could benefit from surface plasmon enhanced absorption. 相似文献