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https://www.readbyqxmd.com/read/29128761/inborn-errors-of-immunity-underlying-fungal-diseases-in-otherwise-healthy-individuals
#1
REVIEW
Juan Li, Donald C Vinh, Jean-Laurent Casanova, Anne Puel
It has been estimated that there are at least 1.5 million fungal species, mostly present in the environment, but only a few of these fungi cause human disease. Most fungal diseases are self-healing and benign, but some are chronic or life-threatening. Acquired and inherited defects of immunity, including breaches of mucocutaneous barriers and circulating leukocyte deficiencies, account for most severe modern-day mycoses. Other types of infection typically accompany these fungal infections. More rarely, severe fungal diseases can strike otherwise healthy individuals...
November 9, 2017: Current Opinion in Microbiology
https://www.readbyqxmd.com/read/29127374/prediction-of-human-prenatal-exposure-to-bisphenol-a-and-bisphenol-a-glucuronide-from-an-ovine-semi-physiological-toxicokinetic-model
#2
Glenn Gauderat, Nicole Picard-Hagen, Pierre-Louis Toutain, Rémi Servien, Catherine Viguié, Sylvie Puel, Marlène Z Lacroix, Tanguy Corbel, Alain Bousquet-Melou, Véronique Gayrard
Bisphenol A (BPA) risk assessment is hampered by the difficulty of determining the extent of internal exposure in the human fetus and uncertainties regarding BPA toxicokinetics (TK) in the maternal-fetal unit. A feto-maternal TK model describing BPA and BPA glucuronide (BPAG) disposition in sheep was humanized, using human TK data obtained after d6-BPA administration on a cookie, to predict BPA and BPAG kinetics in the human mother-fetus unit. Validation of the model predictions included the assessed dose proportionality of BPA and BPAG disposition and the similarity between the simulated and measured time courses of BPA and BPAG in fetal rhesus monkeys after BPA maternal dosing...
November 10, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29055528/development-of-an-on-line-solid-phase-extraction-ultra-high-performance-liquid-chromatography-technique-coupled-to-tandem-mass-spectrometry-for-quantification-of-bisphenol-s-and-bisphenol-s-glucuronide-applicability-to-toxicokinetic-investigations
#3
Flore Grandin, Nicole Picard-Hagen, Véronique Gayrard, Sylvie Puel, Catherine Viguié, Pierre-Louis Toutain, Laurent Debrauwer, Marlène Z Lacroix
Regulatory measures and public concerns regarding bisphenol A (BPA) have led to its replacement by structural analogues, such as Bisphenol S (BPS), in consumer products. At present, no toxicokinetic investigations have been conducted to assess the factors determining human internal exposure to BPS for subsequent risk assessment. Toxicokinetic studies require reliable analytical methods to measure the plasma concentrations of BPS and its main conjugated metabolite, BPS-glucuronide (BPS-G). An efficient on-line SPE-UPLC-MS/MS method for the simultaneous quantification of BPS and BPS-G in ovine plasma was therefore developed and validated in accordance with the European Medicines Agency guidelines for bioanalytical method validation...
December 1, 2017: Journal of Chromatography. A
https://www.readbyqxmd.com/read/29054602/stat1-gain-of-function-and-stat3-loss-of-function-mutations-impair-il-17-and-il-22-immunity-associated-with-cmc
#4
Julia Hiller, Beate Hagl, Renate Effner, Anne Puel, Martin Schaller, Birgit Mascher, Stefanie Eyerich, Kilian Eyerich, Annette F Jansson, Johannes Ring, Jean-Laurent Casanova, Ellen D Renner, Claudia Traidl-Hoffmann
No abstract text is available yet for this article.
October 17, 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/28988713/otx2-genetically-modified-retinal-pigment-epithelial-cells-rescue-photoreceptors-after-transplantation
#5
Christo Kole, Laurence Klipfel, Ying Yang, Vanessa Ferracane, Frederic Blond, Sacha Reichman, Géraldine Millet-Puel, Emmanuelle Clérin, Najate Aït-Ali, Delphine Pagan, Hawa Camara, Marie-Noëlle Delyfer, Emeline F Nandrot, Jose-Alain Sahel, Olivier Goureau, Thierry Léveillard
Inherited retinal degenerations are blinding diseases characterized by the loss of photoreceptors. Their extreme genetic heterogeneity complicates treatment by gene therapy. This has motivated broader strategies for transplantation of healthy retinal pigmented epithelium to protect photoreceptors independently of the gene causing the disease. The limited clinical benefit for visual function reported up to now is mainly due to dedifferentiation of the transplanted cells that undergo an epithelial-mesenchymal transition...
September 8, 2017: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/28832893/cognitive-complaint-in-early-parkinson-s-disease-a-pilot-study
#6
J Dupouy, F Ory-Magne, C Mekies, V Rousseau, M Puel, K Rerat, J Pariente, C Brefel-Courbon
BACKGROUND: Subjective cognitive complaint (SCC) is a criterion recommended by the Movement Disorder Society (MDS) task force for the diagnosis of mild cognitive impairment (MCI). Until now there were few specific tools for detecting SCC in PD. We sought to develop a new tool to assess SCC specifically dedicated for PD. MATERIALS AND METHODS: We set a group of experts in movements disorders and neurocognition to develop an easy-to-use tool based on a visual analogue scale (VAS) for five cognitive domains: memory, executive functions, spatial orientation, attention, and language...
August 22, 2017: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/28830793/piperine-inhibits-aflatoxin-b1-production-in-aspergillus-flavus-by-modulating-fungal-oxidative-stress-response
#7
Isaura Caceres, Rhoda El Khoury, Sylviane Bailly, Isabelle P Oswald, Olivier Puel, Jean-Denis Bailly
Aspergillus flavus, a soil-borne pathogen, represents a danger for humans and animals since it produces the carcinogenic mycotoxin Aflatoxin B1 (AFB1). Approaches aiming the reduction of this fungal contaminant mainly involve chemicals that may also be toxic. Therefore, identification and characterization of natural anti-aflatoxigenic products represents a sustainable alternative strategy. Piperine, a major component of black and long peppers, has been previously demonstrated asan AFB1-inhibitor; nevertheless its mechanism of action was yet to be elucidated...
October 2017: Fungal Genetics and Biology: FG & B
https://www.readbyqxmd.com/read/28760565/familiarity-and-recollection-vs-representational-models-of-medial-temporal-lobe-structures-a-single-case-study
#8
Emilie Lacot, Stéphane Vautier, Stefan Kőhler, Jérémie Pariente, Chris B Martin, Michèle Puel, Jean-Albert Lotterie, Emmanuel J Barbeau
Although it is known that medial temporal lobe (MTL) structures support declarative memory, the fact these structures have different architectonics and circuitry suggests they may also play different functional roles. Selective lesions of MTL structures offer an opportunity to understand these roles. We report, in this study, on JMG, a patient who presents highly unusual lesions that completely affected all MTL structures except for the right hippocampus and parts of neighbouring medial parahippocampal cortex...
September 2017: Neuropsychologia
https://www.readbyqxmd.com/read/28683293/a-specific-gut-microbiota-dysbiosis-of-type-2-diabetic-mice-induces-glp-1-resistance-through-an-enteric-no-dependent-and-gut-brain-axis-mechanism
#9
Estelle Grasset, Anthony Puel, Julie Charpentier, Xavier Collet, Jeffrey E Christensen, François Tercé, Rémy Burcelin
No abstract text is available yet for this article.
July 5, 2017: Cell Metabolism
https://www.readbyqxmd.com/read/28679735/hematopoietic-stem-cell-transplantation-in-29-patients-hemizygous-for-hypomorphic-ikbkg-nemo-mutations
#10
Charline Miot, Kohsuke Imai, Chihaya Imai, Anthony J Mancini, Zeynep Yesim Kucuk, Tokomki Kawai, Ryuta Nishikomori, Etsuro Ito, Isabelle Pellier, Sophie Dupuis Girod, Jeremie Rosain, Shinya Sasaki, Shanmuganathan Chandrakasan, Jana Pachlopnik Schmid, Tsubasa Okano, Estelle Colin, Alberto Olaya-Vargas, Marco Yamazaki-Nakashimada, Waseem Qasim, Sara Espinosa Padilla, Andrea Jones, Alfons Krol, Nyree Cole, Stephen Jolles, Jack Bleesing, Thomas Vraetz, Andrew R Gennery, Mario Abinun, Tayfun Güngör, Beatriz Costa-Carvalho, Antonio Condino-Neto, Paul Veys, Steven M Holland, Gulbu Uzel, Despina Moshous, Benedicte Neven, Stéphane Blanche, Stephan Ehl, Rainer Döffinger, Smita Y Patel, Anne Puel, Jacinta Bustamante, Erwin W Gelfand, Jean-Laurent Casanova, Jordan S Orange, Capucine Picard
X-linked recessive ectodermal dysplasia with immunodeficiency is a rare primary immunodeficiency caused by hypomorphic mutations of the IKBKG gene encoding the nuclear factor κB essential modulator (NEMO) protein. This condition displays enormous allelic, immunological, and clinical heterogeneity, and therapeutic decisions are difficult because NEMO operates in both hematopoietic and nonhematopoietic cells. Hematopoietic stem cell transplantation (HSCT) is potentially life-saving, but the small number of case reports available suggests it has been reserved for only the most severe cases...
September 21, 2017: Blood
https://www.readbyqxmd.com/read/28622416/rosacea-and-demodicidosis-associated-with-gain-of-function-mutation-in-stat1
#11
Julie Second, Anne-Sophie Korganow, Sarah Jannier, Anne Puel, Dan Lipsker
We report a family in which 3 of 5 children as well as the mother, had a gain-of-function (GOF) mutation in the signal transducer and activator of transcription (STAT1) associated with early-onset rosacea and demodicidosis. The proband case (Fig. 1) was a 13-year-old boy of non-consanguineous parents, known for a hypothyroidism, failure to thrive and chronic mucocutaneous candidiasis (CMC) since he was 5-6 years old. On examination, he had medio-facial erythema with telangiectasia, as well as sometimes pruritic papulopustular lesions of the face and neck...
June 16, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28597685/a-gain-of-function-mutation-of-stat1-a-novel-genetic-factor-contributing-to-chronic-mucocutaneous-candidiasis
#12
Narges Eslami, Marzieh Tavakol, Mehrnaz Mesdaghi, Mohammad Gharegozlou, Jean-Laurent Casanova, Anne Puel, Satoshi Okada, Saba Arshi, Mohammad Hassan Bemanian, Morteza Fallahpour, Rasool Molatefi, Farhad Seif, Samaneh Zoghi, Nima Rezaei, Mohammad Nabavi
Heterozygous gain-of-function (GOF) mutations in the signal transducer and activator of transcription 1 (STAT1) have increasingly been identified as a genetic cause of autosomal-dominant (AD) chronic mucocutaneous candidiasis (CMC). In this article, we describe a 33-year-old man who experienced chronic refractory candidiasis, recurrent otitis media, and pneumonia resulting in bronchiectasis, severe oral and esophageal candidiases with strictures associated with hypothyroidism and immune hemolytic anemia. His son also suffered from persistent candidiasis, chronic diarrhea, poor weight gain, and pneumonia that resulted in his demise because of sepsis...
June 1, 2017: Acta Microbiologica et Immunologica Hungarica
https://www.readbyqxmd.com/read/28597146/human-i%C3%AE%C2%BAb%C3%AE-gain-of-function-a-severe-and-syndromic-immunodeficiency
#13
REVIEW
Bertrand Boisson, Anne Puel, Capucine Picard, Jean-Laurent Casanova
Germline heterozygous gain-of-function (GOF) mutations of NFKBIA, encoding IκBα, cause an autosomal dominant (AD) form of anhidrotic ectodermal dysplasia with immunodeficiency (EDA-ID). Fourteen unrelated patients have been reported since the identification of the first case in 2003. All mutations enhanced the inhibitory activity of IκBα, by preventing its phosphorylation on serine 32 or 36 and its subsequent degradation. The mutation certainly or probably occurred de novo in 13 patients, whereas it was inherited from a parent with somatic mosaicism in one patient...
July 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28522685/correction-self-reactive-vh4-34-expressing-igg-b-cells-recognize-commensal-bacteria
#14
Jean-Nicolas Schickel, Salomé Glauzy, Yen-Shing Ng, Nicolas Chamberlain, Christopher Massad, Isabelle Isnardi, Nathan Katz, Gulbu Uzel, Steven M Holland, Capucine Picard, Anne Puel, Jean-Laurent Casanova, Eric Meffre
No abstract text is available yet for this article.
July 3, 2017: Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28521160/evidence-for-bisphenol-a-induced-disruption-of-maternal-thyroid-homeostasis-in-the-pregnant-ewe-at-low-level-representative-of-human-exposure
#15
Davy Guignard, Véronique Gayrard, Marlène Z Lacroix, Sylvie Puel, Nicole Picard-Hagen, Catherine Viguié
Many uncertainties remain regarding the potential of bisphenol A (BPA) as a thyroid disruptor in mammals and the relevance of experimental data to humans. The relevance of the exposure schemes used in experimental in vivo studies is also a major source of uncertainty when analysing the risk of BPA exposure for human health. In this context, the goals of our study, conducted in an ovine model relevant to human gestation and thyroid physiologies, were to: 1) determine the equivalence of subcutaneous and dietary exposures and 2) determine if environmentally relevant doses of BPA can alter gestational and newborn thyroid functions...
September 2017: Chemosphere
https://www.readbyqxmd.com/read/28503543/irak4-deficiency-in-a-patient-with-recurrent-pneumococcal-infections-case-report-and-review-of-the-literature
#16
Karina Gobin, Mary Hintermeyer, Bertrand Boisson, Maya Chrabieh, Pegah Gandil, Ann Puel, Capucine Picard, Jean-Laurent Casanova, John Routes, James Verbsky
Primary immunodeficiencies are genetic defects of the innate or adaptive immune system, resulting in a propensity to infections. The innate immune system is the first line of defense against pathogens and is critical to recognize microbes and start the inflammatory cascade. Sensing of microbes occurs by a number of pathogen-recognition receptors, resulting in the activation of inflammatory signal transduction pathways, such as the activation of NF-κB. Herein, we describe a case of IRAK4 deficiency, a key signal transduction molecule of toll-like and IL-1 receptors...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28500047/self-reactive-vh4-34-expressing-igg-b-cells-recognize-commensal-bacteria
#17
Jean-Nicolas Schickel, Salomé Glauzy, Yen-Shing Ng, Nicolas Chamberlain, Christopher Massad, Isabelle Isnardi, Nathan Katz, Gulbu Uzel, Steven M Holland, Capucine Picard, Anne Puel, Jean-Laurent Casanova, Eric Meffre
The germline immunoglobulin (Ig) variable heavy chain 4-34 (VH4-34) gene segment encodes in humans intrinsically self-reactive antibodies that recognize I/i carbohydrates expressed by erythrocytes with a specific motif in their framework region 1 (FWR1). VH4-34-expressing clones are common in the naive B cell repertoire but are rarely found in IgG memory B cells from healthy individuals. In contrast, CD27(+)IgG(+) B cells from patients genetically deficient for IRAK4 or MYD88, which mediate the function of Toll-like receptors (TLRs) except TLR3, contained VH4-34-expressing clones and showed decreased somatic hypermutation frequencies...
July 3, 2017: Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28467926/a-specific-gut-microbiota-dysbiosis-of-type-2-diabetic-mice-induces-glp-1-resistance-through-an-enteric-no-dependent-and-gut-brain-axis-mechanism
#18
Estelle Grasset, Anthony Puel, Julie Charpentier, Xavier Collet, Jeffrey E Christensen, François Tercé, Rémy Burcelin
Glucagon-like peptide-1 (GLP-1)-based therapies control glycemia in type 2 diabetic (T2D) patients. However, in some patients the treatment must be discontinued, defining a state of GLP-1 resistance. In animal models we identified a specific set of ileum bacteria impairing the GLP-1-activated gut-brain axis for the control of insulin secretion and gastric emptying. Using prediction algorithms, we identified bacterial pathways related to amino acid metabolism and transport system modules associated to GLP-1 resistance...
May 2, 2017: Cell Metabolism
https://www.readbyqxmd.com/read/28453536/rtfa-controls-development-secondary-metabolism-and-virulence-in-aspergillus-fumigatus
#19
Ryan R Myers, Timothy D Smith, Sherine F Elsawa, Olivier Puel, Souraia Tadrist, Ana M Calvo
Invasive aspergillosis by Aspergillus fumigatus is a leading cause of infection-related mortality in immune-compromised patients. In order to discover potential genetic targets to control A. fumigatus infections we characterized rtfA, a gene encoding a putative RNA polymerase II transcription elongation factor-like protein. Our recent work has shown that the rtfA ortholog in the model fungus Aspergillus nidulans regulates morphogenesis and secondary metabolism. The present study on the opportunistic pathogen A...
2017: PloS One
https://www.readbyqxmd.com/read/28365859/epithelial-mesenchymal-transition-and-collective-and-individual-cell-migration-regulate-epithelial-changes-in-the-amikacin-damaged-organ-of-corti
#20
Sabine Ladrech, Michel Eybalin, Jean-Luc Puel, Marc Lenoir
Characterizing the microenvironment of a damaged organ of Corti and identifying the basic mechanisms involved in subsequent epithelial reorganization are critical for improving the outcome of clinical therapies. In this context, we studied the expression of a variety of cell markers related to cell shape, cell adhesion and cell plasticity in the rat organ of Corti poisoned with amikacin. Our results indicate that, after severe outer hair cell losses, the cytoarchitectural reorganization of the organ of Corti implicates epithelial-mesenchymal transition mechanisms and involves both collective and individual cell migratory processes...
August 2017: Histochemistry and Cell Biology
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