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181.
182.
Shuing Kong Hervé Locrelle Adamah Amouzougan Delphine Denarie Philippe Collet Béatrice Pallot-Prades Thierry Thomas Hubert Marotte 《Joint, bone, spine : revue du rhumatisme》2018,85(5):569-572
Objectives
Bone alterations at the subchondral level during rheumatoid arthritis (RA) remain under investigation. It remains unknown whether subchondral bone damage might still occur in RA patients in clinical remission, which could then infer suggesting that even minor subclinical inflammatory changes in the joint can induce local bone loss.Methods
Thirty-two RA patients treated with biological disease-modifying anti-rheumatic drugs (bDMARDs) with low disease activity since at least 6 months and having erosion on the second or third metacarpeal head were enrolled in this pilot cross-sectional study. They were divided in two groups according to local inflammation assessed by Doppler-ultrasound exam surrounding the site of erosion. Cortical and trabecular parameters of the metacarpeal head were then assessed by high-resolution peripheral quantitative computed tomography (HR-pQCT) and compared in both groups.Results
Twenty and twelve RA patients were enrolled in the “Doppler positive erosion” (DE + ) group and Doppler negative erosion (DE?) group, respectively. No difference was observed in their clinical or biological RA characteristics. Both cortical density and thickness were similar among groups. Within the trabecular network, while no difference in bone volume was observed, trabecular density as well as trabecular number were decreased (P < 0.001 and P < 0.05 respectively), whereas trabecular separation and distribution of trabecular separation were increased in DE+ compared to DE? (P < 0.05).Conclusion
In RA patients in low disease activity under bDMARDs, persistence of local inflammation was associated with alteration of the trabecular compartment. Trabecular density was the most strongly altered parameter and could be a candidate to assess drug effect on periarticular bone damage. 相似文献183.
Delphine J. Lee Amila Abeyratne Dennis A. Carson Maripat Corr 《The Journal of experimental medicine》1998,187(3):433-438
The majority of T cell responses are restricted to peptide antigens bound by polymorphic major histocompatibility complex (MHC) molecules. However, peptide antigens can be presented to T cells by murine non-MHC–encoded CD1d (mCD1) molecules, and human T cell lines specific for nonpeptide antigens presented on CD1 isoforms have been identified. It is shown here that antigen-specific, mCD1-restricted lymphocytes can be generated in vivo by immunizing mice with a combination of plasmids encoding chicken ovalbumin, murine CD1d, and costimulatory molecules. Splenocytes from immunized mice have CD1d-restricted, MHC- unrestricted, ovalbumin-specific cytolytic activity that can be inhibited by anti-CD1 antibodies as well as a competing CD1-binding peptide. These results suggest a physiologic role for murine CD1d to present exogenous protein antigens. 相似文献
184.
Arnault Tauzide-Espariat Philipp Sievers Frdrique Larousserie Joseph Benzakoun Delphine Guillemot Gaëlle Pierron Mathilde Duchesne Emmanuelle Uro-Coste Alexandre Roux Alexandre Vasiljevic Tanguy Fenouil David Meyronet Karima Mokhtari Marc Polivka Audrey Rousseau Frdrique Bost-Bezeaud Samir Akoury Johan Pallud Chiara Benevello Lauren Hasty Albane Gareton Emmanule Lechapt Fabrice Chrtien Thomas Blauwblomme Kvin Beccaria Stphanie Puget Felix Sahm Pascale Varlet 《Brain pathology (Zurich, Switzerland)》2022,32(1):e13010
185.
WHIM syndromes with different genetic anomalies are accounted for by impaired CXCR4 desensitization to CXCL12 总被引:8,自引:1,他引:8
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Balabanian K Lagane B Pablos JL Laurent L Planchenault T Verola O Lebbe C Kerob D Dupuy A Hermine O Nicolas JF Latger-Cannard V Bensoussan D Bordigoni P Baleux F Le Deist F Virelizier JL Arenzana-Seisdedos F Bachelerie F 《Blood》2005,105(6):2449-2457
The WHIM syndrome is a rare immunodeficiency disorder characterized by warts, hypogammaglobulinemia, infections, and myelokathexis. Dominant heterozygous mutations of the gene encoding CXCR4, a G-protein-coupled receptor with a unique ligand, CXCL12, have been associated with this pathology. We studied patients belonging to 3 different pedigrees. Two siblings inherited a CXCR4 mutation encoding a novel C-terminally truncated receptor. Two unrelated patients were found to bear a wild-type CXCR4 open reading frame. Circulating lymphocytes and neutrophils from all patients displayed similar functional alterations of CXCR4-mediated responses featured by a marked enhancement of G-protein-dependent responses. This phenomenon relies on the refractoriness of CXCR4 to be both desensitized and internalized in response to CXCL12. Therefore, the aberrant dysfunction of the CXCR4-mediated signaling constitutes a common biologic trait of WHIM syndromes with different causative genetic anomalies. Responses to other chemokines, namely CCL4, CCL5, and CCL21, were preserved, suggesting that, in clinical forms associated with a wild-type CXCR4 open reading frame, the genetic anomaly might target an effector with some degree of selectivity for the CXCL12/CXCR4 axis. We propose that the sustained CXCR4 activity in patient cells accounts for the immune-hematologic clinical manifestations and the profusion of warts characteristic of the WHIM syndrome. 相似文献
186.
The kinase domain of mitochondrial PINK1 faces the cytoplasm 总被引:4,自引:0,他引:4
Zhou C Huang Y Shao Y May J Prou D Perier C Dauer W Schon EA Przedborski S 《Proceedings of the National Academy of Sciences of the United States of America》2008,105(33):12022-12027
Mutations in PTEN-induced putative kinase 1 (PINK1) are a cause of autosomal recessive familial Parkinson's disease (PD). Efforts in deducing the PINK1 signaling pathway have been hindered by controversy around its subcellular and submitochondrial localization and the authenticity of its reported substrates. We show here that this mitochondrial protein exhibits a topology in which the kinase domain faces the cytoplasm and the N-terminal tail is inside the mitochondria. Although deletion of the transmembrane domain disrupts this topology, common PD-linked PINK1 mutations do not. These results are critical in rectifying the location and orientation of PINK1 in mitochondria, and they should help decipher its normal physiological function and potential pathogenic role in PD. 相似文献
187.
Isabelle Laffont Claude Dumas Delphine Pozzi Maria Ruquet Anne Claire Tissier Frédéric Lofaso Olivier Dizien 《Journal of rehabilitation medicine》2007,39(5):399-404
OBJECTIVE: The aim of this study was to evaluate a speech synthesizer with respect to patterns of use and satisfaction, during a 2-month trial at home, and the usefulness of the word prediction function. DESIGN: Prospective study. PARTICIPANTS: Of the 24 patients with severe dysarthria recruited, 10 completed the study. Five patients had cerebral palsy, 3 amyotrophic lateral sclerosis, one locked-in syndrome, and one anoxic brain damage. Mean age was 32 (standard deviation 21) years (range 9-66 years). METHODS: Each participant received 10 hours of training with the device (Dialo) and then used it at home for 2 months. The main outcome measures were: level of use recorded by the device, Quebec User Evaluation of Satisfaction with Assistive Technology (QUEST) satisfaction score (maximum = 5), and time needed to take dictations of standard-dictionary and personal-dictionary words with and without word prediction. RESULTS: Level of use varied widely across participants. Overall satisfaction at the end of the home trial was high, with a mean QUEST score of 3.4 (SD 1) and was related to the level of use of the device. Level of satisfaction at the end of the training session could not predict the level of use at home. No significant differences were found in dictation-taking times with and without word prediction. However, 6 of the 10 patients took dictation faster with than without word prediction. CONCLUSION: This study provides the first evidence supporting the benefits of a speech synthesizer used at home for several weeks. Word prediction is useful for some patients even if increase in dictation speed did not reach significance. 相似文献
188.
Alain Meyer Hlne Chifflot Emmanuel Chatelus Jean‐Franois Kleinmann Ccile Ronde‐Ousteau Delphine Klein Jrmy Jgu Bernard Geny Sandrine Hirshi Matthieu Canuet Gilles Blaison Pierre Kieffer Dan Lipsker Thierry Martin Erik Sauleau Michel Velten Jean Sibilia 《Arthritis \u0026amp; Rheumatology》2016,68(7):1731-1737
189.
190.
Delphine Farlay Gérard Panczer Christian Rey Pierre D. Delmas Georges Boivin 《Journal of bone and mineral metabolism》2010,28(4):433-445
The purpose of this study was to test the hypothesis that mineral maturity and crystallinity index are two different characteristics
of bone mineral. To this end, Fourier transform infrared microspectroscopy (FTIRM) was used. To test our hypothesis, synthetic
apatites and human bone samples were used for the validation of the two parameters using FTIRM. Iliac crest samples from seven
human controls and two with skeletal fluorosis were analyzed at the bone structural unit (BSU) level by FTIRM on sections 2–4 μm
thick. Mineral maturity and crystallinity index were highly correlated in synthetic apatites but poorly correlated in normal
human bone. In skeletal fluorosis, crystallinity index was increased and maturity decreased, supporting the fact of separate
measurement of these two parameters. Moreover, results obtained in fluorosis suggested that mineral characteristics can be
modified independently of bone remodeling. In conclusion, mineral maturity and crystallinity index are two different parameters
measured separately by FTIRM and offering new perspectives to assess bone mineral traits in osteoporosis. 相似文献