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排序方式: 共有5708条查询结果,搜索用时 15 毫秒
71.
Ravel Jean-Marie Benkirane Mehdi Calmels Nadège Marelli Cecilia Ory-Magne Fabienne Ewenczyk Claire Halleb Yosra Tison François Lecocq Claire Pische Guillaume Casenave Philippe Chaussenot Annabelle Frismand Solène Tyvaert Louise Larrieu Lise Pointaux Morgane Drouot Nathalie Bossenmeyer-Pourié Carine Oussalah Abderrahim Guéant Jean-Louis Leheup Bruno Bonnet Céline Anheim Mathieu Tranchant Christine Lambert Laëtitia Chelly Jamel Koenig Michel Renaud Mathilde 《Journal of neurology》2021,268(5):1927-1937
Journal of Neurology - STUB1 has been first associated with autosomal recessive (SCAR16, MIM# 615768) and later with dominant forms of ataxia (SCA48, MIM# 618093). Pathogenic variations in STUB1... 相似文献
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Lamotte Guillaume Takahashi Makoto Wu Tianxia Sullivan Patricia Cherup Jamie Holmes Courtney Goldstein David S. 《Clinical autonomic research》2021,31(4):543-551
Clinical Autonomic Research - Patients with neurogenic orthostatic hypotension in the setting of Lewy body diseases (LBnOH) typically have baroreflex failure and peripheral noradrenergic... 相似文献
73.
Lu Bai Mohammad Webhi Guillaume Dolphijn Bruno Amduri Jean‐Franois Gohy 《Macromolecular chemistry and physics.》2021,222(1)
Solid polymer electrolytes are prepared by mixing various amounts of lithium bis(trifluoromethanesulfonyl)imide with poly(vinylidene fluoride‐co‐vinyl dimethyl phosphonate) statistical copolymers with different compositions. Such copolymers are obtained by conventional radical copolymerization of vinylidene fluoride (VDF) with vinyl dimethyl phosphonate (VDMP) initiated by peroxides. A morphological study of the obtained solid polymer electrolytes (SPEs) shows that only samples prepared from the copolymer with the lower amount of VDMP (16 mol%) result in the formation of homogeneous electrolytes while aggregates of lithium salts are observed for the other copolymers. The best ionic conductivity values are accordingly observed for the copolymers with the lower VDMP amount and are reaching 5 × 10?3 mS cm?1 at 100 °C. The dependence of the ionic conductivity versus temperature suggests that the ionic conductivity is controlled by the motion of polymer segments. Indeed, the ionic conductivity can be increased by adding a small amount of trimethylphosphate plasticizer and can reach 1.9 × 10?2 mS cm?1 at 20 °C. Finally, the prepared SPEs exhibit a high electrochemical stability and a good resistance to flame because of the presence of fire‐retardant phosphate groups in their structure. 相似文献
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Fast imaging strategies for mouse kidney perfusion measurement with pseudocontinuous arterial spin labeling (pCASL) at ultra high magnetic field (11.75 tesla) 下载免费PDF全文
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Combined MEK Inhibition and BMP2 Treatment Promotes Osteoblast Differentiation and Bone Healing in Nf1Osx−/− Mice 下载免费PDF全文
Jean de la Croix Ndong David M Stevens Guillaume Vignaux Sasidhar Uppuganti Daniel S Perrien Xiangli Yang Jeffry S Nyman Eva Harth Florent Elefteriou 《Journal of bone and mineral research》2015,30(1):55-63
Neurofibromatosis type I (NF1) is an autosomal dominant disease with an incidence of 1/3000, caused by mutations in the NF1 gene, which encodes the RAS/GTPase‐activating protein neurofibromin. Non‐bone union after fracture (pseudarthrosis) in children with NF1 remains a challenging orthopedic condition to treat. Recent progress in understanding the biology of neurofibromin suggested that NF1 pseudarthrosis stems primarily from defects in the bone mesenchymal lineage and hypersensitivity of hematopoietic cells to TGFβ. However, clinically relevant pharmacological approaches to augment bone union in these patients remain limited. In this study, we report the generation of a novel conditional mutant mouse line used to model NF1 pseudoarthrosis, in which Nf1 can be ablated in an inducible fashion in osteoprogenitors of postnatal mice, thus circumventing the dwarfism associated with previous mouse models where Nf1 is ablated in embryonic mesenchymal cell lineages. An ex vivo–based cell culture approach based on the use of Nf1flox/flox bone marrow stromal cells showed that loss of Nf1 impairs osteoprogenitor cell differentiation in a cell‐autonomous manner, independent of developmental growth plate–derived or paracrine/hormonal influences. In addition, in vitro gene expression and differentiation assays indicated that chronic ERK activation in Nf1‐deficient osteoprogenitors blunts the pro‐osteogenic property of BMP2, based on the observation that only combination treatment with BMP2 and MEK inhibition promoted the differentiation of Nf1‐deficient osteoprogenitors. The in vivo preclinical relevance of these findings was confirmed by the improved bone healing and callus strength observed in Nf1osx?/? mice receiving Trametinib (a MEK inhibitor) and BMP2 released locally at the fracture site via a novel nanoparticle and polyglycidol‐based delivery method. Collectively, these results provide novel evidence for a cell‐autonomous role of neurofibromin in osteoprogenitor cells and insights about a novel targeted approach for the treatment of NF1 pseudoarthrosis. © 2014 American Society for Bone and Mineral Research. 相似文献
80.
Seth Woodruff Guillaume Feugère Paula Abreu Joseph Heissler Marcia T. Ruiz Frank Jen 《Journal of thrombosis and thrombolysis》2016,42(4):494-504
Venous thromboembolism (VTE) is a common and serious complication in patients with cancer; treatment guidelines recommend extended therapy of ≥6 months with low-molecular-weight heparin (LMWH) for treatment and prevention of recurrent VTE (rVTE) in this population. This post hoc analysis used data from the CLOT study—a phase III, randomized, open-label, controlled study (N = 676)—to compare the efficacy and safety of dalteparin, a LMWH, versus vitamin K antagonist (VKA) for prevention of rVTE in patients with cancer and renal impairment (creatinine clearance <60 ml/min). Overall, 162/676 (24 %) patients had renal impairment at baseline. Patients received subcutaneous dalteparin 200 IU/kg once daily during month 1, followed by 150 IU/kg once daily for months 2–6; or VKA once daily for 6 months, with initial overlapping subcutaneous dalteparin 200 IU/kg once daily for ≥5 days until international normalized ratio was 2.0–3.0 for 2 consecutive days. Endpoints included the rates of rVTE (primary) and bleeding events. Overall, fewer dalteparin-treated patients (2/74 [2.7 %]) experienced ≥1 adjudicated symptomatic rVTE compared with VKA-treated patients (15/88 [17.0 %]; hazard ratio = 0.15 [95 % confidence interval 0.03–0.65]; p = 0.01). Bleeding event rates for both treatments were similar (p = 0.47). In summary, compared with VKA, dalteparin significantly reduced risk of rVTE in patients with cancer and renal impairment (p = 0.01) while exhibiting a comparable safety profile. This analysis supports dosing patients with renal impairment in accordance with patients with normal renal function; however, anti-Xa monitoring could be considered to further support safety in selected patients, particularly those with very severe renal impairment. 相似文献