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259. Hepatocyte nuclear factor 1 inactivation results in hepatic dysfunction, phenylketonuria, and renal Fanconi syndrome

Author

Pontoglio, Marco, Barra, Jacqueline, Hadchouel, Michelle, Doyen, Antonia, Kress, Chantal, Bach, Josephine Poggi, Babinet, Charles, and Yaniv, Moshe

Subjects
Liver cells -- Genetic aspects, Genetic transcription -- Research, Fanconi's anemia -- Genetic aspects, Biological sciences
Abstract

Mice without hepatocyte nuclear factor 1 (HNF1) die during weaning after a progressive wasting syndrome and enlarged kidneys. The transcription rate of genes such as albumin and alpha-1-antitrypsin is decreased while the gene coding for phenylalanine hydroxylase is totally inactivated, causing phenylketonuria. These mice also have Fanconi syndrome because of the malfunction of the renal proximal tube.

Published
1996

260. Developmental and tissue-specific regulation of the murine cardiac actin gene in vivo depends on distinct skeletal and cardiac muscle-specific enhancer elements in addition to the proximal promoter

Author

Biben, Christine, Hadchouel, Juliette, Tajbakhsh, Shahragim, and Buckingham, Margaret

Subjects
Actin -- Genetic aspects, Heart muscle -- Genetic aspects, Genetically modified mice -- Research, Biological sciences
Abstract

Transgenic experiments were carried out to determine the role of the cardiac actin gene in muscle development in the mouse. High-level expression of the cardiac actin gene in the skeletal muscle of transgenic mice involved distal and proximal enhancer sequences and the proximal promoter sequences. It also generated an nlacZ reporter gene. It is suggested that expression of the cardiac actin gene requires a cardiac enhancer located in between the proximal enhancer and proximal promoter regions.

Published
1996

266. Acute genetic ablation of pendrin lowers blood pressure in mice

Author

Philine Wangemann, Régine Chambrey, Dominique Eladari, Francesco Trepiccione, Véronique Baudrie, Jacques Teulon, Juliette Hadchouel, Andrew J. Griffith, Bruno O. Villoutreix, Yoon Byung Choi, Christelle Soukaseum, Nicolas Cornière, Yusuke Kumai, Trepiccione, Francesco, Soukaseum, Christelle, Baudrie, Veronique, Kumai, Yusuke, Teulon, Jacque, Villoutreix, Bruno, Cornière, Nicola, Wangemann, Philine, Griffith, Andrew J, Byung Choi, Yoon, Hadchouel, Juliette, Chambrey, Regine, Eladari, Dominique, Paris-Centre de Recherche Cardiovasculaire (PARCC (UMR_S 970/ U970)), Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Centre de Recherche des Cordeliers (CRC (UMR_S_1138 / U1138)), École pratique des hautes études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Université Paris Cité (UPCité), Molécules Thérapeutiques in silico (MTI), Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre hospitalier Félix-Guyon [Saint-Denis, La Réunion], Kansas State University, and National Institutes of Health [Bethesda] (NIH)

Subjects
Male, Genetically modified mouse, medicine.medical_specialty, [SDV.MHEP.PHY] Life Sciences [q-bio]/Human health and pathology/Tissues and Organs [q-bio.TO], Anion Transport Proteins, 030232 urology & nephrology, Blood Pressure, Mice, Transgenic, 030204 cardiovascular system & hematology, Chloride, Mice, 03 medical and health sciences, 0302 clinical medicine, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, In vivo, Internal medicine, Extracellular fluid, [SDV.MHEP.PHY]Life Sciences [q-bio]/Human health and pathology/Tissues and Organs [q-bio.TO], medicine, otorhinolaryngologic diseases, Animals, Diuretic, Pendrin, Transplantation, Kidney, biology, business.industry, Reabsorption, Original Articles, diuretics, Connecting tubule, [SDV.MHEP.CSC] Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, Endocrinology, Blood pressure, medicine.anatomical_structure, Sulfate Transporters, Nephrology, intercalated cells, Hypertension, biology.protein, business, Intercalated cell
Abstract

International audience; Background: Pendrin, the chloride/bicarbonate exchanger of β-intercalated cells of the renal connecting tubule and the collecting duct, plays a key role in NaCl reabsorption by the distal nephron. Therefore, pendrin may be important for the control of extracellular fluid volume and blood pressure.Methods: Here, we have used a genetic mouse model in which the expression of pendrin can be switched-on in vivo by the administration of doxycycline. Pendrin can also be rapidly removed when doxycycline administration is discontinued. Therefore, our genetic strategy allows us to test selectively the acute effects of loss of pendrin function.Results: We show that acute loss of pendrin leads to a significant decrease of blood pressure. In addition, acute ablation of pendrin did not alter significantly the acid-base status or blood K + concentration.Conclusion: By using a transgenic mouse model, avoiding off-target effects related to pharmacological compounds, this study suggests that pendrin could be a novel target to treat hypertension.

Published
2017
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267. Double Knockout of the Na + -Driven Cl − /HCO 3 − Exchanger and Na + /Cl − Cotransporter Induces Hypokalemia and Volume Depletion

Author

Régine Chambrey, Christian A. Hübner, Anne Sinning, Maximilien Jayat, Karen I. López-Cayuqueo, Stéphanie Baron, R. Todd Alexander, Nicolas Cornière, Juliette Hadchouel, Dominique Eladari, Nikita Radionov, Francesco Trepiccione, Friedrich-Schiller-Universität = Friedrich Schiller University Jena [Jena, Germany], Paris-Centre de Recherche Cardiovasculaire (PARCC (UMR_S 970/ U970)), Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPCité), Université Paris Descartes - Faculté de Médecine (UPD5 Médecine), Université Paris Descartes - Paris 5 (UPD5), Centro de Estudios Científicos (CECs), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Centre Hospitalier Universitaire de La Réunion (CHU La Réunion), University of Alberta, Centre National de la Recherche Scientifique (CNRS), Hadchouel, Juliette, Sinning, Anne, Radionov, Nikita, Trepiccione, Francesco, López Cayuqueo, Karen I, Jayat, Maximilien, Baron, Stéphanie, Cornière, Nicola, Alexander, R. Todd, Eladari, Dominique, Hübner, Christian A, and Chambrey, Régine

Subjects
0301 basic medicine, Epithelial sodium channel, medicine.medical_specialty, hypertension, [SDV.MHEP.PHY] Life Sciences [q-bio]/Human health and pathology/Tissues and Organs [q-bio.TO], diuretic, 030232 urology & nephrology, Mice, 03 medical and health sciences, 0302 clinical medicine, Downregulation and upregulation, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, In vivo, Internal medicine, medicine, hypokalemia, [SDV.MHEP.PHY]Life Sciences [q-bio]/Human health and pathology/Tissues and Organs [q-bio.TO], Animals, Chloride-Bicarbonate Antiporters, Thiazide, Mice, Knockout, Blood Volume, biology, Animal, Chemistry, Chloride-Bicarbonate Antiporter, General Medicine, Pendrin, Hypokalemia, diuretics, Up-Regulation, [SDV.MHEP.CSC] Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, Basic Research, 030104 developmental biology, Endocrinology, Nephrology, biology.protein, medicine.symptom, Cotransporter, Homeostasis, medicine.drug
Abstract

International audience; We recently described a novel thiazide-sensitive electroneutral NaCl transport mechanism resulting from the parallel operation of the Cl-/HCO3- exchanger pendrin and the Na+-driven Cl-/2HCO3- exchanger (NDCBE) in β-intercalated cells of the collecting duct. Although a role for pendrin in maintaining Na+ balance, intravascular volume, and BP is well supported, there is no in vivo evidence for the role of NDCBE in maintaining Na+ balance. Here, we show that deletion of NDCBE in mice caused only subtle perturbations of Na+ homeostasis and provide evidence that the Na+/Cl- cotransporter (NCC) compensated for the inactivation of NDCBE. To unmask the role of NDCBE, we generated Ndcbe/Ncc double-knockout (dKO) mice. On a normal salt diet, dKO and single-knockout mice exhibited similar activation of the renin-angiotensin-aldosterone system, whereas only dKO mice displayed a lower blood K+ concentration. Furthermore, dKO mice displayed upregulation of the epithelial sodium channel (ENaC) and the Ca2+-activated K+ channel BKCa. During NaCl depletion, only dKO mice developed marked intravascular volume contraction, despite dramatically increased renin activity. Notably, the increase in aldosterone levels expected on NaCl depletion was attenuated in dKO mice, and single-knockout and dKO mice had similar blood K+ concentrations under this condition. In conclusion, NDCBE is necessary for maintaining sodium balance and intravascular volume during salt depletion or NCC inactivation in mice. Furthermore, NDCBE has an important role in the prevention of hypokalemia. Because NCC and NDCBE are both thiazide targets, the combined inhibition of NCC and the NDCBE/pendrin system may explain thiazide-induced hypokalemia in some patients.

Published
2017
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269. Pneumo Pédiatrie : l'asthme de l'enfant

Author

S. Blanchon, C. Mordacq, Antoine Deschildre, S. Lejeune, Alice Hadchouel, C. Thumerelle, and A. Carsin

Subjects
Pulmonary and Respiratory Medicine, Gynecology, 03 medical and health sciences, medicine.medical_specialty, 0302 clinical medicine, 030228 respiratory system, business.industry, medicine, 030212 general & internal medicine, business, Article
Published
2016
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270. Atteintes pulmonaires au cours de la drépanocytose chez l’enfant

Author

A.-A. Lopes, Alice Hadchouel, Z. Germont, A. Massiot, L. Calamy, C. Thivent, J. Tencer, A. Lemoine, and T. Pincez

Subjects
03 medical and health sciences, 0302 clinical medicine, 030220 oncology & carcinogenesis, Pediatrics, Perinatology and Child Health, 030215 immunology
Abstract

Resume Le poumon est la cible de complications aigues et chroniques dans la drepanocytose. L’hypoxie chronique est frequente, elle joue un role dans certaines de ces complications et peut etre deletere a long terme. Sa prise en charge repose davantage sur le programme transfusionnel que l’oxygenotherapie. Le syndrome thoracique aigu (STA) peut etre d’installation tres rapide et demeure la principale cause de deces premature. Principalement secondaire a une infection ou une crise vaso-occlusive (CVO), sa prevention et son traitement sont essentiels et ont fait l’objet de nouvelles recommandations. L’asthme n’est pas plus frequent chez les enfants drepanocytaires mais pourrait etre associe a la survenue de CVO, de STA, voire de deces precoces. Sa prise en charge n’est pas specifique mais l’utilisation des corticoides systemiques doit etre prudente car elle n’est pas denuee de risques. L’hypertension pulmonaire chez l’enfant drepanocytaire est probablement source de comorbidite mais pas de mortalite contrairement a ce qui est constate chez l’adulte. Son depistage est systematique mais sa prise en charge n’est pas etablie. Concernant les fonctions respiratoires, si la prevalence du syndrome obstructif est faible, celle du syndrome restrictif augmente avec l’âge, temoignant d’un declin rapide et precoce des volumes pulmonaires. L’endurance des enfants drepanocytaires est plus faible mais ne contre-indique pas la pratique mesuree du sport. Les troubles du sommeil, dont le syndrome d’apnee obstructive du sommeil, sont plus frequents. Ils necessitent un depistage regulier car ils peuvent etre source de troubles neurologiques significativement diminues par leur prise en charge. Cette importante morbi-mortalite justifie une surveillance respiratoire attentive chez ces enfants.

Published
2016
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271. With no lysine L-WNK1 isoforms are negative regulators of the K+-Cl− cotransporters

Author

Gerardo Gamba, María Chávez-Canales, Paola de los Heros, Norma Vázquez, Adrián Rafael Murillo-de-Ozores, Adriana Mercado, Erika Moreno, Zesergio Melo, Silvana Bazúa-Valenti, and Juliette Hadchouel

Subjects
0301 basic medicine, Physiology, Xenopus, Protein Serine-Threonine Kinases, Transfection, Minor Histocompatibility Antigens, Xenopus laevis, 03 medical and health sciences, Osmoregulation, WNK Lysine-Deficient Protein Kinase 1, Animals, Humans, Protein Isoforms, Solute Carrier Family 12, Member 2, Protein Interaction Domains and Motifs, Phosphorylation, Cell Size, Symporters, biology, urogenital system, Lysine, Intracellular Signaling Peptides and Proteins, Articles, Cell Biology, biology.organism_classification, WNK1, WNK4, HEK293 Cells, 030104 developmental biology, Biochemistry, Mutation, Symporter, Signal transduction, Cotransporter, Intracellular, Signal Transduction
Abstract

The K+-Cl− cotransporters (KCC1-KCC4) encompass a branch of the SLC12 family of electroneutral cation-coupled chloride cotransporters that translocate ions out of the cell to regulate various factors, including cell volume and intracellular chloride concentration, among others. L-WNK1 is an ubiquitously expressed kinase that is activated in response to osmotic stress and intracellular chloride depletion, and it is implicated in two distinct hereditary syndromes: the renal disease pseudohypoaldosteronism type II (PHAII) and the neurological disease hereditary sensory neuropathy 2 (HSN2). The effect of L-WNK1 on KCC activity is unknown. Using Xenopus laevis oocytes and HEK-293 cells, we show that the activation of KCCs by cell swelling was prevented by L-WNK1 coexpression. In contrast, the activity of the Na+-K+-2Cl− cotransporter NKCC1 was remarkably increased with L-WNK1 coexpression. The negative effect of L-WNK1 on the KCCs is kinase dependent. Elimination of the STE20 proline-alanine rich kinase (SPAK)/oxidative stress-responsive kinase (OSR1) binding site or the HQ motif required for the WNK-WNK interaction prevented the effect of L-WNK1 on KCCs, suggesting a required interaction between L-WNK1 molecules and SPAK. Together, our data support that NKCC1 and KCCs are coordinately regulated by L-WNK1 isoforms.

Published
2016
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272. Réabsorption du sel et sécrétion du potassium par le néphron distal

Author

Juliette Hadchouel, Chloé Rafael, and Maria Chavez-Canales

Subjects
0301 basic medicine, Protein-Serine-Threonine Kinases, urogenital system, Reabsorption, Kinase, General Medicine, Biology, WNK1, Distal nephron, Monogenic disease, Molecular biology, General Biochemistry, Genetics and Molecular Biology, WNK4, 03 medical and health sciences, 030104 developmental biology, 0302 clinical medicine, Cotransporter, 030217 neurology & neurosurgery
Abstract

The study of Familial Hyperkalemic Hypertension (FHHt), a rare monogenic disease, allowed remarkable advances in the understanding of the mechanisms of regulation of NaCl reabsorption by the distal nephron. FHHt results from mutations in the genes encoding WNK1 and WNK4, two serine-threonine kinases of the WNK (With No lysine [K]) family. The clinical manifestations of FHHt are due, among others, to an increased activity of the Na(+)-Cl(-) cotransporter NCC. Several groups therefore tried to understand how WNK1 and WNK4 could regulate NCC. However, the data were often contradictory. Two of our recent studies allowed to partially explain these controversies and to propose a new model for the regulation of NCC by the WNKs.

Published
2016
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276. Alveolar proteinosis of genetic origins

Author

Christophe Delacourt, Alice Hadchouel, Rola Abou Taam, Jacques de Blic, David Drummond, M. Lebourgeois, Service de Pneumologie et d'Allergologie Pédiatriques, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Institut Mondor de Recherche Biomédicale (IMRB), Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR10-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Université Paris Descartes - Paris 5 (UPD5), Université Paris Cité (UPCité), Service de Pneumologie Allergologie [CHU Necker], Centre de Recherche des Cordeliers (CRC (UMR_S_1138 / U1138)), École pratique des hautes études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Université Paris Cité (UPCité), Health data- and model- driven Knowledge Acquisition (HeKA), Inria de Paris, Institut National de Recherche en Informatique et en Automatique (Inria)-Institut National de Recherche en Informatique et en Automatique (Inria)-Centre de Recherche des Cordeliers (CRC (UMR_S_1138 / U1138)), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Université Paris Cité (UPCité)-École pratique des hautes études (EPHE), Centre Référence des Maladies Héréditaires du Métabolisme de l'Enfant et de l'Adulte [CHU Necker] (MaMEA Necker), and CHU Necker - Enfants Malades [AP-HP]

Subjects
Adult, lcsh:RC705-779, 0301 basic medicine, Pulmonary and Respiratory Medicine, business.industry, Alveolar proteinosis, Infant, lcsh:Diseases of the respiratory system, Chronic interstitial lung disease, Pulmonary Alveolar Proteinosis, 030105 genetics & heredity, medicine.disease, Bioinformatics, Birt–Hogg–Dubé syndrome, Birt-Hogg-Dube Syndrome, 03 medical and health sciences, 030104 developmental biology, Humans, Medicine, Child, business, Pulmonary alveolar proteinosis, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology, Exome sequencing
Abstract

International audience; Pulmonary alveolar proteinosis (PAP) is a rare form of chronic interstitial lung disease, characterised by the intra-alveolar accumulation of lipoproteinaceous material. Numerous conditions can lead to its development. Whereas the autoimmune type is the main cause in adults, genetic defects account for a large part of cases in infants and children. Even if associated extra-respiratory signs may guide the clinician during diagnostic work-up, next-generation sequencing panels represent an efficient diagnostic tool. Exome sequencing also allowed the discovery of new variants and genes involved in PAP. The aim of this article is to summarise our current knowledge of genetic causes of PAP.

Published
2020
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278. Effectiveness and safety of ruxolitinib for the treatment of refractory systemic idiopathic juvenile arthritis like associated with interstitial lung disease : a case report

Author

Vivien Béziat, Jean-Laurent Casanova, Laureline Berteloot, Romain Levy, L. Polivka, Alice Hadchouel, and Brigitte Bader-Meunier

Subjects
musculoskeletal diseases, 0301 basic medicine, medicine.medical_specialty, Ruxolitinib, Immunology, Arthritis, General Biochemistry, Genetics and Molecular Biology, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Tocilizumab, Rheumatology, Prednisone, Internal medicine, Nitriles, Pulmonary fibrosis, medicine, Humans, Immunology and Allergy, Leukocytosis, 030203 arthritis & rheumatology, business.industry, Interstitial lung disease, medicine.disease, Arthritis, Juvenile, Pyrimidines, 030104 developmental biology, chemistry, Macrophage activation syndrome, Pyrazoles, Immunotherapy, medicine.symptom, Lung Diseases, Interstitial, business, medicine.drug
Abstract

We read with interest the report of a series of 61 patients with systemic juvenile idiopathic arthritis (s-JIA) or s-JIA-like associated with high-fatality lung diseases.1 Lung disease was associated with digital clubbing, a high frequency of anaphylactic reactions to tocilizumab, and macrophage activation syndrome (MAS). Because of the low 5-year survival probability of 42%, there is an urgent need to identify efficient drugs to treat such patients. Herein we report the effectiveness and safety of ruxolitinib in one patient demonstrating clinical and radiological characteristics consistent to those reported by Saper et al A 2-year-old girl born to non-consanguineous parents presented with s-JIA-like since the age of 12 months, including recurring episodes of unexplained fever, urticaria, arthralgia, poor general health status, leukocytosis and elevated serum C-reactive protein (CRP). The use of corticosteroids resulted in a complete remission but the patient relapsed when prednisone was tapered below 0.5 mg/kg/day. Owing to this corticosteroid dependence, the patient received several lines of biological agents from the …

Published
2020
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279. Incidence of cirrhosis in children with chronic hepatitis

Author

Vajro, Pietro, Hadchouel, Paul, Hadchouel, Michelle, Bernard, Olivier, and Alagille, Daniel

Subjects
Hepatitis in children -- Complications, Liver cirrhosis -- Causes of, Liver cirrhosis -- Diagnosis, Health
Abstract

Cirrhosis, a chronic liver disease, can follow inflammatory liver disease (hepatitis), and is associated with complications such as liver failure, cancer, and cardiopulmonary complications. There is currently no definitive technique to diagnose cirrhosis. Blind liver needle biopsies, in which the liver is not viewed, have not been diagnostically effective. As a result, the true frequency of cirrhosis following chronic hepatitis is unclear. The effectiveness of combined laparoscopy (visual exploration of the abdomen) with needle biopsy was investigated in 92 children (64 female) aged 1 to 16 years with chronic hepatitis. Cirrhosis was found in 15 children with hepatitis B antigen (a protein indicative of hepatitis B infection), in one case, cirrhosis was not suspected by laparoscopic findings. Onset of cirrhotic symptoms, significant enlargement of the spleen, or loss of a hepatitis B protein from the blood were more frequently, but not consistently, found in cirrhotic patients. Cirrhosis was present in 41 of 46 patients with autoimmune (antibodies formed against one's own tissues) hepatitis. African or Asian family origins were a common factor in cirrhotic patients. Long-term follow-up of an average of 7.5 years showed that patients without cirrhosis remained well. Of patients with cirrhosis, 12 died, 7 others developed serious complications, and 5 received or were awaiting liver transplantation. Analysis of the extent of cirrhosis suggested that cirrhosis may be a part of chronic hepatitis from the outset, rather than the endpoint of disease. The effective diagnosis provided by the combination of laparoscopy plus biopsy allows proper treatment and follow-up care of children with chronic hepatitis. (Consumer Summary produced by Reliance Medical Information, Inc.)

Published
1990

282. Contributors

Author

Abbasi, Soraya, Afshar, Yalda, Ahn, Sun-Young, Albertine, Kurt H., Allegaert, Karel, Alper, Seth L., Altit, Gabriel, Alvaro, Ruben E., Alvira, Cristina M., Maia Amorim, Natália Carlos, Anbuhl, Kelsey L., Andersen, Claus Yding, Anderson, Richard A., Andrews, Katrina A., Askenazi, David J., Fernandes Assunção, Débora Gabriela, Auten, Richard Lambert, Jr., Autmizguine, Julie, Azhibekov, Timur, Back, Stephen A., Bahr, Timothy M., Baker, Peter Russell, II, Bancalari, Eduardo H., Barichello, Tatiana, Battaglia, Frederick C., Bauer, Andrew J., Baum, Michel, Bavis, Ryan W., Beardsall, Kathryn, Beggs, Simon, Benchimol, Corinne, Benders, Manon J.N.L., Bennet, Laura, Bennett, Phillip R., Berger, Melvin, Bernhard, Wolfgang, Bertram, John F., Bhombal, Shazia, Bhutani, Vinod K., Black, Mary Jane, Bliss, Joseph M., Bolender, David L., Bowdin, Sarah C., Boyd, Scott D., Brandenburg, Joline E., Brown, Laura D., Burrin, Douglas G., Cannon, Barbara, Caplan, Michael, Carlson, Susan E., Carlton, David P., Chaemsaithong, Piya, Chang, Jill, Charlton, Jennifer R., Chemtob, Sylvain, Chheda, Sadhana, Childs, Andrew J., Chu, David H., Chung, Wendy K., Cilio, Maria Roberta, Clark, David A., Clarke, Paul, Cleal, Jane K., Clemente, Ethel G., Clements, John A., Clyman, Ronald I., Cohen, Jennifer L., Cohen, Susan S., Collins, Amélie, Collodel, Allan, Colombo, John, Combes, Alexander N., Copp, Andrew J., Cotten, C. Michael, Crawford, Peter A., Crowe, James E., Jr., Crowther, C.A., Cullen-McEwen, Luise A., Cutfield, Wayne S., Cyr-Depauw, Chanèle, Danielly da S. Ribeiro, Karla, Dauby, Nicolas, Davenport, Patricia, Davidson, Joanne O., Cruz, Diomel de la, Gomes de Oliveira, Priscila, de Vrijer, Barbra, Del Colle, Andrew, Delacourt, Christophe, Diacovo, Thomas G., Disdier, Clémence, Dormans, John P., Duhamel, François, Duong, Minh Dien, Dysart, Kevin, Eichenwald, Eric C., El-Khuffash, Afif F., Ivor Ellis, Peter James, Empey, Kerry M., Ercal, Baris, Erdős, Melinda, Errede, Mariella, Feldman, Brian J., Fidanza, Mario, Fogarty, Matthew J., Friedlich, Philippe S., Fujiwara, Ryoichi, Gallo, Vittorio, Ganguly, Abhrajit, Gao, Yuansheng, Garland, Marianne, Geddes, Donna, Dip (Sci), Post Grad, Georgieff, Michael K., Gien, Jason, Giussani, Dino A., Goldman, Armond S., Gomez-Lopez, Nardhy, Good, Misty, Good, Pamela I., Gordon, Scott M., Green, Lucy R., Greene, Nicholas D.E., Gridneva, Zoya, Grigoriou, Emmanouil, Grimberg, Adda, Grunau, Ruth E., Guignard, Jean-Pierre, Gunn, Alistair J., Gurtunca, Nursen, Gustafson, Kathleen M., Hadchouel, Alice, Haddad, Gabriel G., Hale, Thomas W., Hambidge, K. Michael, Hammerman, Cathy, Ruud Hansen, Thor Willy, Hanson, Mark A., Harbeson, Danny, Harding, J.E., Harding, Richard, Harris, Mary Catherine, Hartmann, Peter, Hartnett, M. Elizabeth, Hasbun, Rodrigo, Haugen, Guttorm, Hawkes, Colin P., Hay, William W., Jr., Heine, Vivi M., Helmrath, Michael A., Hendricks-Muñoz, Karen D., Herrera, Emilio, Hiatt, Michael J., Hooper, Stuart B., Hooven, Thomas A., Iacobelli, Silvia, Inder, Terrie E., Iruela-Arispe, M. Luisa, Jadcherla, Sudarshan Rao, Jain, Deepak, Jetton, Jennifer G., Jobe, Alan H., Jones, Helen, Jose, Pedro A., Jung, Eunjung, Kallapur, Suhas G., Kaplan, Michael, Karumanchi, S. Ananth, Kaskel, Frederick J., Levitt Katz, Lorraine E., Kavus, Haluk, Keeney, Susan E., Kern, Steven E., Khanjani, Shirin, Khlevner, Julie, Kilpatrick, Laurie E., Kim, Chang-Ryul, Kingma, Paul S., Kinsella, John P., Kiserud, Torvid, Koenig, Joyce M., Kohli, Rohit, Kollmann, Tobias R., Kolls, Jay K., Konecny, Christina M., Kratimenos, Panagiotis, Krebs, Nancy F., Krutsch, Kaytlin, Kuhn-Riordon, Kara, Kulik, Thomas J., Kumar, T. Rajendra, Kutikov, Jessica Katz, Lakshminrusimha, Satyan, Lasunción, Miguel Angel, Lavoie, Pascal M., Lawrence, Shelley M., Lee, Mark K., Lee, Mary M., Lee, Yvonne K., Leibel, Sandra L., Levy, Ofer, Levy, Philip T., Lewis, Rohan M., Li, Changgong, Lin, Fangming, Lobritto, Steven, Loomis, Cynthia A., MacFarlane, Peter M., MacIntyre, David A., Mahe, Maxime M., Mamsen, Linn Salto, Mankouski, Anastasiya, Mantilla, Carlos B., Marchant, Arnaud, Margolis, Kara Gross, Maródi, László, Maršál, Karel, Martin, Richard J., Martin Carli, Jayne F., Martinez, Hugo R., Matsell, Douglas G., Matthews, Dwight E., McArdle, Harry J., McKinlay, C.J.D., McManaman, James L., McNamara, Patrick J., Meschia, Giacomo, Mestan, Karen, Miller, Steven P., Minoo, Parviz, Mir, Imran N., Mitchell, Lisa J., Mižíková, Ivana, Mizuno, Tomoyuki, Momper, Jeremiah D., Monagle, Paul, Monks, Jenifer, Mortola, Jacopo P., Muglia, Louis J., Munshi, Upender K., Narasimhan, Sumana, Narendran, Vivek, Nedergaard, Jan, Nelin, Leif D., Neu, Josef, Nielsen, Sandra C.A., Nigam, Sanjay K., Nizet, Victor, Nogee, Lawrence M., Noori, Shahab, Norris, Andrew W., O’Brien, Barbara M., O’Brien, Lori L., O’Callaghan, Karen M., Ogilvy-Stuart, Amanda, Ohls, Robin K., Ortega-Senovilla, Henar, O’Sullivan, Justin M., Panitch, Howard B., Penn, Anna A., Penn, Raymond B., Petroff, Margaret G., Philipps, Anthony F., Pisani, Francesco, Pleasure, David, Pomeroy, Scott L., Post, Martin, Prakash, Y.S., Prozialeck, Joshua D., Pysher, Theodore J., Quigley, Raymond, Rabinovitch, Marlene, Raffay, Thomas M., Raj, J. Usha, Ramsey, Laura B., Rana, Sarosh, Randis, Tara M., Ranger, Manon, Regnault, Timothy R.H., Rios, Danielle R., Romero, Roberto, Rosenfeld, Charles R., Ross, A. Catharine, Roth, Daniel E., Rozycki, Henry J., Ryan, Thomas D., Sahni, Rakesh, Sarnat, Harvey B., Satlin, Lisa M., Scafidi, Joseph, Schellpfeffer, Michael A., Schierding, William, Schwartz, George J., Segar, Jeffrey L., Selewski, David T., Seri, Istvan, Shaffer, Thomas H., Shearer, Martin J., Shroyer, Noah F., Sieck, Gary C., Simmons, Rebecca A., Singh, Neel Kamal, Sivieri, Emidio, Smith, Laura, Smyth, Ian M., Sola-Visner, Martha C., Solhaug, Michael J., Sperandio, Markus, Sperling, Mark A., Srinivasan, Lakshmi, Stanescu, Diana E., Stanley, Charles A., Steinhorn, Robin H., Stinson, Lisa, Stonestreet, Barbara S., Strasburger, Janette F., Styne, Dennis M., Sun, Xin, Sussel, Lori, Tam, Emily W.Y., Tan, Libo, te Pas, Arjan B., Ten, Vadim S., Thébaud, Bernard, Thornton, Claire, Tollin, Daniel J., Towbin, Jeffrey A., Truog, William E., III, Uhler, Kristin M., van den Akker, Chris H.P., Nicolaas van den Anker, John, van den Hoff, Maurice J.B., van Goudoever, Johannes (Hans) B., Vickers, Mark H., Vinks, Alexander A., Virgintino, Daniela, Visscher, Marty O., Vonderohe, Caitlin E., Vyas, Neha V., Wacker-Gussmann, Annette, Walch, Abby, Wallace, Megan J., Walsh, Brian H., Wambach, Jennifer A., Wang, Linda X., Warburton, David, Ward, Robert M., Watt, Kevin M., Watterberg, Kristi L., Werner, Lynne A., Wernimont, Sarah A., Wershil, Barry K., Wessels, Andy, Whitsett, Jeffrey A., Willems, Fabienne, Win, Myat Su, Wohlmuth, Christoph, Wolf, Matthias T., Wolfson, Marla R., Wong, Ronald J., Wynn, James L., Yeo, Lami, Yoder, Bradley A., Yuskaitis, Christopher J., Zabinsky, Jennifer, and Zhou, Dan

Published
2022
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283. Association between asthma and lung function in adolescents born very preterm: results of the EPIPAGE cohort study

Author

Catherine Arnaud, Jessica Rousseau, Laure Couderc, Alice Hadchouel, Jean-Christophe Rozé, Adèle Bellino, Christophe Delacourt, Marie Mittaine, Stéphane Marret, Pierre-Yves Ancel, Marie Verstraete, Didier Pinquier, Institut Mondor de recherche biomédicale (IMRB), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Laboratoire sols, solides, structures - risques [Grenoble] (3SR ), Institut polytechnique de Grenoble - Grenoble Institute of Technology (Grenoble INP )-Centre National de la Recherche Scientifique (CNRS)-Université Grenoble Alpes [2016-2019] (UGA [2016-2019]), CIC Plurithématique de Nantes, Institut National de la Santé et de la Recherche Médicale (INSERM)-Ministère des Affaires sociales et de la Santé-Direction générale de l'offre de soins (DGOS)-Centre hospitalier universitaire de Nantes (CHU Nantes), INSERM, U1027, Toulouse, France., Service de pédiatrie médicale et médecine de l'adolescent [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Team 4 'NeoVasc' - INSERM U1245, Génomique et Médecine Personnalisée du Cancer et des Maladies Neuropsychiatriques (GPMCND), Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Aix Marseille Université (AMU), Recherche Epidémiologique en Santé Périnatale et Santé des Femmes et des Enfants (UMR_S 953), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Descartes - Paris 5 (UPD5)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris-Sud - Paris 11 (UP11), Domaines Océaniques (LDO), Institut national des sciences de l'Univers (INSU - CNRS)-Université de Brest (UBO)-Observatoire des Sciences de l'Univers-Institut d'écologie et environnement-Centre National de la Recherche Scientifique (CNRS), Team 4 NeoVasc - Region Team ERI 28 INSERM (Neovasc), Université Paris-Sud - Paris 11 (UP11)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Epidémiologie et analyses en santé publique : risques, maladies chroniques et handicaps (LEASP), Université Toulouse III - Paul Sabatier (UT3), Université de Toulouse (UT)-Université de Toulouse (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut Necker Enfants-Malades (INEM - UM 111 (UMR 8253 / U1151)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Cité (UPCité), Laboratoire sols, solides, structures - risques [Grenoble] (3SR), Université Joseph Fourier - Grenoble 1 (UJF)-Institut polytechnique de Grenoble - Grenoble Institute of Technology (Grenoble INP)-Institut National Polytechnique de Grenoble (INPG)-Centre National de la Recherche Scientifique (CNRS)-Université Grenoble Alpes (UGA), and Centre National de la Recherche Scientifique (CNRS)-Institut d'écologie et environnement-Observatoire des Sciences de l'Univers-Université de Brest (UBO)-Institut national des sciences de l'Univers (INSU - CNRS)

Subjects
Male, 0301 basic medicine, Pulmonary and Respiratory Medicine, Pediatrics, medicine.medical_specialty, Adolescent, Term Birth, [SDV]Life Sciences [q-bio], 03 medical and health sciences, Sex Factors, 0302 clinical medicine, Forced Expiratory Volume, Humans, Medicine, Prospective Studies, Respiratory system, Child, ComputingMilieux_MISCELLANEOUS, Lung function, Bronchopulmonary Dysplasia, Respiratory Sounds, Asthma, business.industry, Confounding, paediatric asthma, medicine.disease, 3. Good health, respiratory tract diseases, Very preterm, 030104 developmental biology, Breath Tests, 030228 respiratory system, Bronchopulmonary dysplasia, Case-Control Studies, Child, Preschool, Cohort, Premature Birth, Female, Nitrogen Oxides, France, business, paediatric lung disaese, Cohort study
Abstract

Prematurity and bronchopulmonary dysplasia (BPD) affect long-term lung function. We studied the respiratory outcome of adolescents born very preterm and controls from the Etude EPIdémiologique sur les Petits Ages Gestationnels cohort and analysed their current lung function in relation to asthma symptoms (categorised in three age groups) from birth. In models including BPD, asthma at each age and confounding factors in the preterm group, BPD and preschool wheeze were the only independent variables associated with FEV1. Preschool wheeze is an independent factor associated with FEV1 impairment in adolescents born very preterm. These results highlight the need for optimal management of early respiratory symptoms in preterm-born infants.Trial registration numberResults, NCT01424553.

Published
2018
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285. Association between asthma and lung function in adolescents born very preterm: results of the EPIPAGE cohort study

Author

Jessica Rousseau, Adèle Bellino, Alice Hadchouel-Duvergé, Stéphane Marret, Christophe Delacourt, Jean-Christophe Rozé, Catherine Arnaud, and Pierre-Yves Ancel

Subjects
Pediatrics, medicine.medical_specialty, business.industry, Asthma symptoms, medicine.disease, Very preterm, Bronchopulmonary dysplasia, Volunteer Sample, medicine, Respiratory system, business, Lung function, Asthma, Cohort study
Abstract

Background: Prematurity and bronchopulmonary dysplasia (BPD) affect long-term lung function. The potential associations between postnatal respiratory symptoms, their course, and lung function are yet to be explored. We aimed to study the respiratory outcome of adolescents born very preterm and analyze their current lung function in relation to asthma symptoms from birth. Methods: Lung function was measured in a volunteer sample of 304 very preterm and 47 term-born adolescents from the prospective EPIPAGE birth cohort. Results were correlated with neonatal data and asthma symptoms collected in EPIPAGE follow-up questionnaires during the preschool (0-5 years old) and school (8 years old) periods, and at adolescence using the ISAAC auto-questionnaire. Results: Preschool wheeze was more frequent in preterm subjects than in controls (p=0.03), and in BPD than in non-BPD preterm subjects (p=0.04). Forced expiratory volume in one second (FEV1) was lower in preterm subjects than in controls (p=0.03), and in BPD than in non-BPD preterm subjects (p Conclusions: Preschool wheeze is an independent factor associated with FEV1 impairment in adolescents born very preterm. These results highlight the need for optimal management of early respiratory symptoms in preterm-born infants.

Published
2018
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286. Successful haematopoietic stem cell transplantation in a case of pulmonary alveolar proteinosis due to GM-CSF receptor deficiency

Author

Cécile Pochon, Christophe Delacourt, Bénédicte Neven, Alessandra Magnani, Alice Hadchouel, Despina Moshous, Coralie Briand, Laureline Berteloot, Alain Fischer, Stéphane Blanche, Cyril Schweitzer, Julie Bruneau, Jacques de Blic, Guilhem Cros, Marie-Louise Frémond, Marina Cavazzana, Cécile Bonnet, and Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy)

Subjects
0301 basic medicine, Pulmonary and Respiratory Medicine, Pathology, medicine.medical_specialty, medicine.medical_treatment, Hematopoietic stem cell transplantation, Pulmonary Alveolar Proteinosis, 03 medical and health sciences, 0302 clinical medicine, Oxygen therapy, medicine, Humans, X chromosome, ComputingMilieux_MISCELLANEOUS, [SDV.MHEP.PED]Life Sciences [q-bio]/Human health and pathology/Pediatrics, medicine.diagnostic_test, business.industry, Hematopoietic Stem Cell Transplantation, Infant, [SDV.MHEP.HEM]Life Sciences [q-bio]/Human health and pathology/Hematology, Hypoxia (medical), medicine.disease, 3. Good health, Transplantation, Haematopoiesis, 030104 developmental biology, Bronchoalveolar lavage, 030228 respiratory system, Receptors, Granulocyte-Macrophage Colony-Stimulating Factor, Female, medicine.symptom, Pulmonary alveolar proteinosis, business
Abstract

Cyril Schweitzer (CS) : A 1.5-year-old girl was referred to the Paediatric Pneumology Department of the University Hospital of Nancy, France, for progressive tachypnoea and hypoxia. She suffered from intrauterine growth retardation, and was born prematurely at 31 weeks’ gestation; birth weight: 1.160 kg (≤2 SD); birth length: 37 cm (≤2 SD). The patient presented with acute respiratory distress syndrome at birth, requiring treatment with a dose of surfactant, 9 days of mechanical ventilation and 8 weeks of oxygen therapy. On arrival to our centre, she was dyspnoeic on exertion and oxygen saturations were between 91% and 94%. Further investigations included a high-resolution CT scan and a bronchoalveolar lavage (BAL). Laureline Berteloot (LB) and Julie Bruneau (JB) : CT imaging of the chest revealed a ‘crazy paving’ pattern whereas analysis of BAL fluid failed to identify any underlying disease. CS : Hypoxia transiently improved after initiation of inhaled corticosteroids and antireflux therapy but relapsed after a few months, so that a second BAL was performed. JB : The second BAL fluid revealed the presence of extracellular lipid and protein deposits and foamy alveolar macrophages. CS and Cecile Bonnet (CeB) : These abnormalities led to a diagnosis of pulmonary alveolar proteinosis (PAP) at the age of 2.6 years, which was confirmed by genetic testing; complex chromosome abnormalities were detected, with (1) deletion of CSF2RA and CRLF2 on the X chromosome inherited from the mother, and (2) a de novo rearrangement of the paternal X chromosome, resulting in loss of both CSF2RA alleles.1 CS, Christophe Delacourt (CD) and Jacques De Blic (JDB) : Recessive mutations in the α or β subunits of the granulocyte macrophage colony-stimulating factor receptor (GM-CSF-R, encoded respectively by CSF2RA and CSF2RB ) cause two subtypes of hereditary PAP.2 Impairment of GM-CSF-dependent surfactant clearance by alveolar macrophages leads to the progressive accumulation of surfactant in the alveolar space …

Published
2018
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287. Mutations in MARS identified in a specific type of pulmonary alveolar proteinosis alter methionyl-tRNA synthetase activity

Author

Jacques de Blic, Marc Mirande, Martine Comisso, Alice Hadchouel, Institut de Biologie Intégrative de la Cellule (I2BC), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Université Paris-Saclay-Centre National de la Recherche Scientifique (CNRS), Département Biologie des Génomes (DBG), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Université Paris-Saclay-Centre National de la Recherche Scientifique (CNRS)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Université Paris-Saclay-Centre National de la Recherche Scientifique (CNRS), Assemblages supramoléculaires et traduction (MARS), Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Université Paris-Saclay-Centre National de la Recherche Scientifique (CNRS)-Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Université Paris-Saclay-Centre National de la Recherche Scientifique (CNRS)-Institut de Biologie Intégrative de la Cellule (I2BC), Service de Pneumologie Allergologie [CHU Necker], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)

Subjects
Models, Molecular, Protein Conformation, alpha-Helical, 0301 basic medicine, RNA, Transfer, Met, pulmonary alveolar proteinosis, [SDV]Life Sciences [q-bio], Genetic Vectors, Mutant, Gene Expression, Aminoacylation, Methionine-tRNA Ligase, Biochemistry, Substrate Specificity, 03 medical and health sciences, chemistry.chemical_compound, Methionine, Escherichia coli, medicine, Animals, Humans, Missense mutation, Protein Interaction Domains and Motifs, Amino Acid Sequence, Translation factor, Cloning, Molecular, aminoacylation kinetics, Molecular Biology, chemistry.chemical_classification, Binding Sites, Sequence Homology, Amino Acid, Chemistry, methionyl-tRNA synthetase, Cell Biology, medicine.disease, Molecular biology, Recombinant Proteins, Cytosol, 030104 developmental biology, Enzyme, Mutation, Mutagenesis, Site-Directed, Protein Conformation, beta-Strand, Transfer RNA Aminoacylation, Pulmonary alveolar proteinosis, Sequence Alignment, Protein Binding
Abstract

International audience; Biallelic missense mutations in MARS are responsible for rare but severe cases of pulmonary alveolar proteinosis (PAP) prevalent on the island of La Réunion. MARS encodes cytosolic methionyl-tRNA synthetase (MetRS), an essential translation factor. The multisystemic effects observed in patients with this form of PAP are consistent with a loss-of-function defect in an ubiquitously expressed enzyme. The pathophysiological mechanisms involved in MARS-related PAP are currently unknown. In this work, we analyzed the effect of the PAP-related mutations in MARS on the thermal stability and on the catalytic parameters of the MetRS mutants, relative to wild-type. The effect of these mutations on the structural integrity of the enzyme as a member of the cytosolic multisynthetase complex was also investigated. Our results establish that the PAP-related substitutions in MetRS impact the tRNAMet -aminoacylation reaction especially at the level of methionine recognition, and suggest a direct link between the loss of activity of the enzyme and the pathological disorders in PAP.

Published
2018
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288. Intégrité Scientifique au CNRS; Vers un dispositif pérenne de promotion des valeurs de l’intégrité scientifique au CNRS, et de traitement des allégations de manquement à l’intégrité

Author

Armengaud, Maïté, Bertrand, Joël, Duee, Pierre-Henri, Hadchouel, Michelle, Kirchner, Claude, Le Gall, Olivier, Moret-Bailly, Joël, Centre National de la Recherche Scientifique (CNRS), Institut National de la Recherche Agronomique (INRA), Institut National de la Santé et de la Recherche Médicale (INSERM), Inria Siège, Institut National de Recherche en Informatique et en Automatique (Inria), Biologie du fruit et pathologie (BFP), Université Bordeaux Segalen - Bordeaux 2-Institut National de la Recherche Agronomique (INRA)-Université Sciences et Technologies - Bordeaux 1, Université Jean Monnet [Saint-Étienne] (UJM), CNRS, Université Bordeaux Segalen - Bordeaux 2-Institut National de la Recherche Agronomique (INRA)-Université Sciences et Technologies - Bordeaux 1 (UB), and Université Jean Monnet - Saint-Étienne (UJM)

Subjects
[SHS.HISPHILSO]Humanities and Social Sciences/History, Philosophy and Sociology of Sciences
Published
2018

291. Anti-MDA5 juvenile idiopathic inflammatory myopathy: a specific subgroup defined by differentially enhanced interferon-α signalling

Author

Melki, Isabelle, primary, Devilliers, Hervé, additional, Gitiaux, Cyril, additional, Bondet, Vincent, additional, Duffy, Darragh, additional, Charuel, Jean-Luc, additional, Miyara, Makoto, additional, Bokov, Plamen, additional, Kheniche, Ahmed, additional, Kwon, Theresa, additional, Authier, François Jérôme, additional, Allenbach, Yves, additional, Belot, Alexandre, additional, Bodemer, Christine, additional, Bourrat, Emmanuelle, additional, Dumaine, Cécile, additional, Fabien, Nicole, additional, Faye, Albert, additional, Frémond, Marie-Louise, additional, Hadchouel, Alice, additional, Kitabayashi, Naoki, additional, Lepelley, Alice, additional, Martin-Niclos, Maria José, additional, Mudumba, Sasi, additional, Musset, Lucile, additional, Quartier, Pierre, additional, Rice, Gillian I, additional, Seabra, Luis, additional, Uettwiller, Florence, additional, Uggenti, Carolina, additional, Viel, Sebastien, additional, Rodero, Mathieu P, additional, Crow, Yanick J, additional, and Bader-Meunier, Brigitte, additional

Published
2019
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