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3. Influx of nitrogen-rich material from the outer Solar System indicated by iron nitride in Ryugu samples

Author

Matsumoto, Toru, Noguchi, Takaaki, Miyake, Akira, Igami, Yohei, Haruta, Mitsutaka, Seto, Yusuke, Miyahara, Masaaki, Tomioka, Naotaka, Saito, Hikaru, Hata, Satoshi, Harries, Dennis, Takigawa, Aki, Nakauchi, Yusuke, Tachibana, Shogo, Nakamura, Tomoki, Matsumoto, Megumi, Ishii, Hope A., Bradley, John P., Ohtaki, Kenta, Dobrică, Elena, Leroux, Hugues, Le Guillou, Corentin, Jacob, Damien, de la Peña, Francisco, Laforet, Sylvain, Marinova, Maya, Langenhorst, Falko, Beck, Pierre, Phan, Thi H. V., Rebois, Rolando, Abreu, Neyda M., Gray, Jennifer, Zega, Thomas, Zanetta, Pierre-M., Thompson, Michelle S., Stroud, Rhonda, Burgess, Kate, Cymes, Brittany A., Bridges, John C., Hicks, Leon, Lee, Martin R., Daly, Luke, Bland, Phil A., Zolensky, Michael E., Frank, David R., Martinez, James, Tsuchiyama, Akira, Yasutake, Masahiro, Matsuno, Junya, Okumura, Shota, Mitsukawa, Itaru, Uesugi, Kentaro, Uesugi, Masayuki, Takeuchi, Akihisa, Sun, Mingqi, Enju, Satomi, Michikami, Tatsuhiro, Yurimoto, Hisayoshi, Okazaki, Ryuji, Yabuta, Hikaru, Naraoka, Hiroshi, Sakamoto, Kanako, Yada, Toru, Nishimura, Masahiro, Nakato, Aiko, Miyazaki, Akiko, Yogata, Kasumi, Abe, Masanao, Okada, Tatsuaki, Usui, Tomohiro, Yoshikawa, Makoto, Saiki, Takanao, Tanaka, Satoshi, Terui, Fuyuto, Nakazawa, Satoru, Watanabe, Sei-ichiro, and Tsuda, Yuichi

Published
2024
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4. Analysis of muscle magnetic resonance imaging of a large cohort of patient with VCP-mediated disease reveals characteristic features useful for diagnosis

Author

Esteller, Diana, Schiava, Marianela, Verdú-Díaz, José, Villar-Quiles, Rocío-Nur, Dibowski, Boris, Venturelli, Nadia, Laforet, Pascal, Alonso-Pérez, Jorge, Olive, Montse, Domínguez-González, Cristina, Paradas, Carmen, Vélez, Beatriz, Kostera-Pruszczyk, Anna, Kierdaszuk, Biruta, Rodolico, Carmelo, Claeys, Kristl, Pál, Endre, Malfatti, Edoardo, Souvannanorath, Sarah, Alonso-Jiménez, Alicia, de Ridder, Willem, De Smet, Eline, Papadimas, George, Papadopoulos, Constantinos, Xirou, Sofia, Luo, Sushan, Muelas, Nuria, Vilchez, Juan J., Ramos-Fransi, Alba, Monforte, Mauro, Tasca, Giorgio, Udd, Bjarne, Palmio, Johanna, Sri, Srtuhi, Krause, Sabine, Schoser, Benedikt, Fernández-Torrón, Roberto, López de Munain, Adolfo, Pegoraro, Elena, Farrugia, Maria Elena, Vorgerd, Mathias, Manousakis, Georgious, Chanson, Jean Baptiste, Nadaj-Pakleza, Aleksandra, Cetin, Hakan, Badrising, Umesh, Warman-Chardon, Jodi, Bevilacqua, Jorge, Earle, Nicholas, Campero, Mario, Díaz, Jorge, Ikenaga, Chiseko, Lloyd, Thomas E., Nishino, Ichizo, Nishimori, Yukako, Saito, Yoshihiko, Oya, Yasushi, Takahashi, Yoshiaki, Nishikawa, Atsuko, Sasaki, Ryo, Marini-Bettolo, Chiara, Guglieri, Michela, Straub, Volker, Stojkovic, Tanya, Carlier, Robert Y., and Díaz-Manera, Jordi

Published
2023
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5. Correction to: Analysis of muscle magnetic resonance imaging of a large cohort of patient with VCP‑mediated disease reveals characteristic features useful for diagnosis

Author

Esteller, Diana, Schiava, Marianela, Verdú-Díaz, José, Villar-Quiles, Rocío-Nur, Dibowski, Boris, Venturelli, Nadia, Laforet, Pascal, Alonso-Pérez, Jorge, Olive, Montse, Domínguez-González, Cristina, Paradas, Carmen, Vélez, Beatriz, Kostera-Pruszczyk, Anna, Kierdaszuk, Biruta, Rodolico, Carmelo, Claeys, Kristl, Pál, Endre, Malfatti, Edoardo, Souvannanorath, Sarah, Alonso-Jiménez, Alicia, de Ridder, Willem, De Smet, Eline, Papadimas, George, Papadopoulos, Constantinos, Xirou, Sofia, Luo, Sushan, Muelas, Nuria, Vilchez, Juan J., Ramos-Fransi, Alba, Monforte, Mauro, Tasca, Giorgio, Udd, Bjarne, Palmio, Johanna, Sri, Srtuhi, Krause, Sabine, Schoser, Benedikt, Fernández-Torrón, Roberto, López de Munain, Adolfo, Pegoraro, Elena, Farrugia, Maria Elena, Vorgerd, Mathias, Manousakis, Georgious, Chanson, Jean Baptiste, Nadaj-Pakleza, Aleksandra, Cetin, Hakan, Badrising, Umesh, Warman-Chardon, Jodi, Bevilacqua, Jorge, Earle, Nicholas, Campero, Mario, Díaz, Jorge, Ikenaga, Chiseko, Lloyd, Thomas E., Nishino, Ichizo, Nishimori, Yukako, Saito, Yoshihiko, Oya, Yasushi, Takahashi, Yoshiaki, Nishikawa, Atsuko, Sasaki, Ryo, Marini-Bettolo, Chiara, Guglieri, Michela, Straub, Volker, Stojkovic, Tanya, Carlier, Robert Y., and Díaz-Manera, Jordi

Published
2024
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6. Elevated TNF-α Leads to Neural Circuit Instability in the Absence of Interferon Regulatory Factor 8.

Author

Feinberg, Philip, Becker, Shannon, Chung, Leeyup, Ferrari, Loris, Stellwagen, David, Anaclet, Christelle, Durán-Laforet, Violeta, Faust, Travis, Sumbria, Rachita, and Schafer, Dorothy

Subjects
IRF8, TNF alpha, microglia, neural-immune, seizures, synapses, Animals, Female, Interferon Regulatory Factors, Male, Mice, Multiple Sclerosis, Seizures, Tumor Necrosis Factor-alpha
Abstract

Interferon regulatory factor 8 (IRF8) is a transcription factor necessary for the maturation of microglia, as well as other peripheral immune cells. It also regulates the transition of microglia and other immune cells to a pro-inflammatory phenotype. Irf8 is also a known risk gene for multiple sclerosis and lupus, and it has recently been shown to be downregulated in schizophrenia. While most studies have focused on IRF8-dependent regulation of immune cell function, little is known about how it impacts neural circuits. Here, we show by RNAseq from Irf8 -/- male and female mouse brains that several genes involved in regulation of neural activity are dysregulated. We then show that these molecular changes are reflected in heightened neural excitability and a profound increase in susceptibility to lethal seizures in male and female Irf8 -/- mice. Finally, we identify that TNF-α is elevated specifically in microglia in the CNS, and genetic or acute pharmacological blockade of TNF-α in the Irf8 -/- CNS rescued the seizure phenotype. These results provide important insights into the consequences of IRF8 signaling and TNF-α on neural circuits. Our data further suggest that neuronal function is impacted by loss of IRF8, a factor involved in neuropsychiatric and neurodegenerative diseases.SIGNIFICANCE STATEMENT Here, we identify a previously unknown and key role for interferon regulator factor 8 (IRF8) in regulating neural excitability and seizures. We further determine that these effects on neural circuits are through elevated TNF-α in the CNS. As IRF8 has most widely been studied in the context of regulating the development and inflammatory signaling in microglia and other immune cells, we have uncovered a novel function. Further, IRF8 is a risk gene for multiple sclerosis and lupus, IRF8 is dysregulated in schizophrenia, and elevated TNF-α has been identified in a multitude of neurologic conditions. Thus, elucidating these IRF8 and TNF-α-dependent effects on brain circuit function has profound implications for understanding underlying, therapeutically relevant mechanisms of disease.

Published
2022

7. Genotype-phenotype correlations in valosin-containing protein disease: a retrospective muticentre study.

Author

Schiava, Marianela, Ikenaga, Chiseko, Villar-Quiles, Rocío, Caballero-Ávila, Marta, Topf, Ana, Nishino, Ichizo, Kimonis, Virginia, Udd, Bjarne, Schoser, Benedikt, Zanoteli, Edmar, Souza, Paulo, Tasca, Giorgio, Lloyd, Thomas, Lopez-de Munain, Adolfo, Paradas, Carmen, Pegoraro, Elena, Nadaj-Pakleza, Aleksandra, De Bleecker, Jan, Badrising, Umesh, Alonso-Jiménez, Alicia, Kostera-Pruszczyk, Anna, Miralles, Francesc, Shin, Jin-Hong, Bevilacqua, Jorge, Olivé, Montse, Vorgerd, Matthias, Kley, Rudi, Brady, Stefen, Williams, Timothy, Domínguez-González, Cristina, Papadimas, George, Warman-Chardon, Jodi, Claeys, Kristl, de Visser, Marianne, Muelas, Nuria, LaForet, Pascal, Malfatti, Edoardo, Alfano, Lindsay, Nair, Sruthi, Manousakis, Georgios, Kushlaf, Hani, Harms, Matthew, Nance, Christopher, Ramos-Fransi, Alba, Rodolico, Carmelo, Hewamadduma, Channa, Cetin, Hakan, García-García, Jorge, Pál, Endre, Farrugia, Maria, Lamont, Phillipa, Quinn, Colin, Nedkova-Hristova, Velina, Peric, Stojan, Luo, Sushan, Oldfors, Anders, Taylor, Kate, Ralston, Stuart, Stojkovic, Tanya, Weihl, Conrad, and Diaz-Manera, Jordi

Subjects
FRONTOTEMPORAL DEMENTIA, GENETICS, INCL BODY MYOSITIS, MUSCLE DISEASE, MYOPATHY
Abstract

BACKGROUND: Valosin-containing protein (VCP) disease, caused by mutations in the VCP gene, results in myopathy, Pagets disease of bone (PBD) and frontotemporal dementia (FTD). Natural history and genotype-phenotype correlation data are limited. This study characterises patients with mutations in VCP gene and investigates genotype-phenotype correlations. METHODS: Descriptive retrospective international study collecting clinical and genetic data of patients with mutations in the VCP gene. RESULTS: Two hundred and fifty-five patients (70.0% males) were included in the study. Mean age was 56.8±9.6 years and mean age of onset 45.6±9.3 years. Mean diagnostic delay was 7.7±6 years. Symmetric lower limb weakness was reported in 50% at onset progressing to generalised muscle weakness. Other common symptoms were ventilatory insufficiency 40.3%, PDB 28.2%, dysautonomia 21.4% and FTD 14.3%. Fifty-seven genetic variants were identified, 18 of these no previously reported. c.464G>A (p.Arg155His) was the most frequent variant, identified in the 28%. Full time wheelchair users accounted for 19.1% with a median time from disease onset to been wheelchair user of 8.5 years. Variant c.463C>T (p.Arg155Cys) showed an earlier onset (37.8±7.6 year) and a higher frequency of axial and upper limb weakness, scapular winging and cognitive impairment. Forced vital capacity (FVC) below 50% was as risk factor for being full-time wheelchair user, while FVC

Published
2022

9. West Nile Virus-Induced Expression of Senescent Gene Lgals3bp Regulates Microglial Phenotype within Cerebral Cortex

Author

Artem Arutyunov, Violeta Durán-Laforet, Shenjian Ai, Loris Ferrari, Robert Murphy, Dorothy P. Schafer, and Robyn S. Klein

Subjects
microglia, neuroinfectious disease, flavivirus encephalitis, neurodegeneration, aging, microglia transcriptomics, Microbiology, QR1-502
Abstract

Microglia, the resident macrophages of the central nervous system, exhibit altered gene expression in response to various neurological conditions. This study investigates the relationship between West Nile Virus infection and microglial senescence, focusing on the role of LGALS3BP, a protein implicated in both antiviral responses and aging. Using spatial transcriptomics, RNA sequencing and flow cytometry, we characterized changes in microglial gene signatures in adult and aged mice following recovery from WNV encephalitis. Additionally, we analyzed Lgals3bp expression and generated Lgals3bp-deficient mice to assess the impact on neuroinflammation and microglial phenotypes. Our results show that WNV-activated microglia share transcriptional signatures with aged microglia, including upregulation of genes involved in interferon response and inflammation. Lgals3bp was broadly expressed in the CNS and robustly upregulated during WNV infection and aging. Lgals3bp-deficient mice exhibited reduced neuroinflammation, increased homeostatic microglial numbers, and altered T cell populations without differences in virologic control or survival. These data indicate that LGALS3BP has a role in regulating neuroinflammation and microglial activation and suggest that targeting LGALS3BP might provide a potential route for mitigating neuroinflammation-related cognitive decline in aging and post-viral infections.

Published
2024
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10. Linking Cause and Effect: Nanoscale Vibrational Spectroscopy of Space Weathering from Asteroid Ryugu

Author

Sylvain Laforet, Corentin Le Guillou, Francisco de la Peña, Michael Walls, Luiz H. G. Tizei, Maya Marinova, Pierre Beck, Van T. H. Phan, Damien Jacob, Bahae-eddine Mouloud, Daniel Hallatt, Mario Pelaez-Fernandez, Jean-Christophe Viennet, David Troadec, Takaaki Noguchi, Toru Matsumoto, Akira Miyake, Hisayoshi Yurimoto, and Hugues Leroux

Subjects
Space weather, Asteroid surfaces, Remote sensing, Small Solar System bodies, Vibrational spectroscopy, Infrared spectroscopy, Astrophysics, QB460-466
Abstract

Airless bodies are subjected to space-weathering effects that modify the first few microns of their surface. Therefore, understanding their impact on the optical properties of asteroids is key to the interpretation of their color variability and infrared reflectance observations. The recent Hayabusa2 sample return mission to asteroid Ryugu offers the first opportunity to study these effects, in the case of the most abundant spectral type among the main-asteroid belt, C-type objects. This study employs vibrational electron energy-loss spectroscopy in the transmission electron microscope to achieve the spatial resolution required to measure the distinct mid-infrared spectral signature of Ryugu's space-weathered surface. The comparison with the spectrum of the pristine underlying matrix reveals the loss of structural -OH and C-rich components in the space-weathered layers, providing direct experimental evidence that exposure to the space environment tends to mask the optical signatures of phyllosilicates and carbonaceous matter. Our findings should contribute to rectifying potential underestimations of water and carbon content of C-type asteroids when studied through remote sensing with new-generation telescopes.

Published
2024
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11. A dehydrated space-weathered skin cloaking the hydrated interior of Ryugu

Author

Noguchi, Takaaki, Matsumoto, Toru, Miyake, Akira, Igami, Yohei, Haruta, Mitsutaka, Saito, Hikaru, Hata, Satoshi, Seto, Yusuke, Miyahara, Masaaki, Tomioka, Naotaka, Ishii, Hope A., Bradley, John P., Ohtaki, Kenta K., Dobrică, Elena, Leroux, Hugues, Le Guillou, Corentin, Jacob, Damien, de la Peña, Francisco, Laforet, Sylvain, Marinova, Maya, Langenhorst, Falko, Harries, Dennis, Beck, Pierre, Phan, Thi H. V., Rebois, Rolando, Abreu, Neyda M., Gray, Jennifer, Zega, Thomas, Zanetta, Pierre-M., Thompson, Michelle S., Stroud, Rhonda, Burgess, Kate, Cymes, Brittany A., Bridges, John C., Hicks, Leon, Lee, Martin R., Daly, Luke, Bland, Phil A., Zolensky, Michael E., Frank, David R., Martinez, James, Tsuchiyama, Akira, Yasutake, Masahiro, Matsuno, Junya, Okumura, Shota, Mitsukawa, Itaru, Uesugi, Kentaro, Uesugi, Masayuki, Takeuchi, Akihisa, Sun, Mingqi, Enju, Satomi, Takigawa, Aki, Michikami, Tatsuhiro, Nakamura, Tomoki, Matsumoto, Megumi, Nakauchi, Yusuke, Abe, Masanao, Arakawa, Masahiko, Fujii, Atsushi, Hayakawa, Masahiko, Hirata, Naru, Hirata, Naoyuki, Honda, Rie, Honda, Chikatoshi, Hosoda, Satoshi, Iijima, Yu-ichi, Ikeda, Hitoshi, Ishiguro, Masateru, Ishihara, Yoshiaki, Iwata, Takahiro, Kawahara, Kousuke, Kikuchi, Shota, Kitazato, Kohei, Matsumoto, Koji, Matsuoka, Moe, Mimasu, Yuya, Miura, Akira, Morota, Tomokatsu, Nakazawa, Satoru, Namiki, Noriyuki, Noda, Hirotomo, Noguchi, Rina, Ogawa, Naoko, Ogawa, Kazunori, Okada, Tatsuaki, Okamoto, Chisato, Ono, Go, Ozaki, Masanobu, Saiki, Takanao, Sakatani, Naoya, Sawada, Hirotaka, Senshu, Hiroki, Shimaki, Yuri, Shirai, Kei, Sugita, Seiji, Takei, Yuto, Takeuchi, Hiroshi, Tanaka, Satoshi, Tatsumi, Eri, Terui, Fuyuto, Tsukizaki, Ryudo, Wada, Koji, Yamada, Manabu, Yamada, Tetsuya, Yamamoto, Yukio, Yano, Hajime, Yokota, Yasuhiro, Yoshihara, Keisuke, Yoshikawa, Makoto, Yoshikawa, Kent, Fukai, Ryohta, Furuya, Shizuho, Hatakeda, Kentaro, Hayashi, Tasuku, Hitomi, Yuya, Kumagai, Kazuya, Miyazaki, Akiko, Nakato, Aiko, Nishimura, Masahiro, Soejima, Hiromichi, Suzuki, Ayako I., Usui, Tomohiro, Yada, Toru, Yamamoto, Daiki, Yogata, Kasumi, Yoshitake, Miwa, Connolly, Jr, Harold C., Lauretta, Dante S., Yurimoto, Hisayoshi, Nagashima, Kazuhide, Kawasaki, Noriyuki, Sakamoto, Naoya, Okazaki, Ryuji, Yabuta, Hikaru, Naraoka, Hiroshi, Sakamoto, Kanako, Tachibana, Shogo, Watanabe, Sei-ichiro, and Tsuda, Yuichi

Published
2023
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14. Endothelial cells regulate astrocyte to neural progenitor cell trans-differentiation in a mouse model of stroke

Author

Wenlu Li, Emiri T. Mandeville, Violeta Durán-Laforet, Norito Fukuda, Zhanyang Yu, Yi Zheng, Aaron Held, Ji-Hyun Park, Takafumi Nakano, Masayoshi Tanaka, Jingfei Shi, Elga Esposito, Wanting Niu, Changhong Xing, Kazuhide Hayakawa, Ignacio Lizasoain, Klaus van Leyen, Xunming Ji, Brian J. Wainger, Maria A. Moro, and Eng H. Lo

Subjects
Science
Abstract

Damaged brains try to repair themselves by producing neurons in areas where neurogenesis does not normally occur. Here, the authors show that brain endothelial cells provide microvesicle-encased signals that convert parenchymal astrocytes into neural progenitors, thus improving outcomes after stroke.

Published
2022
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15. Prognosis of Right Ventricular Systolic Dysfunction in Patients With Duchenne Muscular Dystrophy

Author

Abdallah Fayssoil, Nicolas Mansencal, Lee S. Nguyen, Olivier Nardi, Rabah Ben Yaou, France Leturcq, Helge Amthor, Karim Wahbi, Henri Marc Becane, Frederic Lofaso, Helene Prigent, Guillaume Bassez, Anthony Behin, Tanya Stojkovic, Bertrand Fontaine, Denis Duboc, Olivier Dubourg, Bernard Clair, Pascal Laforet, Djillali Annane, and David Orlikowski

Subjects
Duchenne muscular dystrophy, mortality, prognosis, right ventricle, ventilation, Diseases of the circulatory (Cardiovascular) system, RC666-701
Abstract

Background Chronic respiratory failure and heart involvement may occur in Duchenne muscular dystrophy. We aimed to assess the prognostic value of the right ventricular (RV) systolic dysfunction in patients with Duchenne muscular dystrophy. Methods and Results We studied 90 genetically proven patients with Duchenne muscular dystrophy from 2010 to 2019, to obtain respiratory function and Doppler echocardiographic RV systolic function. Prognostic value was assessed in terms of death and cardiac events. The median age was 27.5 years, and median forced vital capacity was at 10% of the predicted value: 83 patients (92%) were on home mechanical ventilation. An RV systolic dysfunction was found in 46 patients (51%). In patients without RV dysfunction at inclusion, a left ventricular systolic dysfunction at inclusion was associated with a higher risk of developing RV dysfunction during follow‐up with an odds ratio of 4.5 (P=0.03). RV systolic dysfunction was significantly associated with cardiac events, mainly acute heart failure (62%) and cardiogenic shock (23%). In a multivariable Cox model, the adjusted hazard ratio was 4.96 (95% CI [1.09–22.6]; P=0.04). In terms of death, we found a significant difference between patients with RV dysfunction versus patients without RV dysfunction in the Kaplan–Meier curves (log‐rank P=0.045). Conclusions RV systolic dysfunction is frequently present in patients with Duchenne muscular dystrophy and is associated with increased risk of cardiac events, irrespective of left ventricular dysfunction and mechanical ventilation. Registration URL: https://www.clinicaltrials.org; unique identifier: NCT02501083.

Published
2023
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20. Muscle cells of sporadic amyotrophic lateral sclerosis patients secrete neurotoxic vesicles

Author

Laura Le Gall, William J. Duddy, Cecile Martinat, Virginie Mariot, Owen Connolly, Vanessa Milla, Ekene Anakor, Zamalou G. Ouandaogo, Stephanie Millecamps, Jeanne Lainé, Udaya Geetha Vijayakumar, Susan Knoblach, Cedric Raoul, Olivier Lucas, Jean Philippe Loeffler, Peter Bede, Anthony Behin, Helene Blasco, Gaelle Bruneteau, Maria Del Mar Amador, David Devos, Alexandre Henriques, Adele Hesters, Lucette Lacomblez, Pascal Laforet, Timothee Langlet, Pascal Leblanc, Nadine Le Forestier, Thierry Maisonobe, Vincent Meininger, Laura Robelin, Francois Salachas, Tanya Stojkovic, Giorgia Querin, Julie Dumonceaux, Gillian Butler Browne, Jose‐Luis González De Aguilar, Stephanie Duguez, and Pierre Francois Pradat

Subjects
Secreted vesicles, Cell–cell communication, MND, sporadic ALS, Diseases of the musculoskeletal system, RC925-935, Human anatomy, QM1-695
Abstract

Abstract Background The cause of the motor neuron (MN) death that drives terminal pathology in amyotrophic lateral sclerosis (ALS) remains unknown, and it is thought that the cellular environment of the MN may play a key role in MN survival. Several lines of evidence implicate vesicles in ALS, including that extracellular vesicles may carry toxic elements from astrocytes towards MNs, and that pathological proteins have been identified in circulating extracellular vesicles of sporadic ALS patients. Because MN degeneration at the neuromuscular junction is a feature of ALS, and muscle is a vesicle‐secretory tissue, we hypothesized that muscle vesicles may be involved in ALS pathology. Methods Sporadic ALS patients were confirmed to be ALS according to El Escorial criteria and were genotyped to test for classic gene mutations associated with ALS, and physical function was assessed using the ALSFRS‐R score. Muscle biopsies of either mildly affected deltoids of ALS patients (n = 27) or deltoids of aged‐matched healthy subjects (n = 30) were used for extraction of muscle stem cells, to perform immunohistology, or for electron microscopy. Muscle stem cells were characterized by immunostaining, RT‐qPCR, and transcriptomic analysis. Secreted muscle vesicles were characterized by proteomic analysis, Western blot, NanoSight, and electron microscopy. The effects of muscle vesicles isolated from the culture medium of ALS and healthy myotubes were tested on healthy human‐derived iPSC MNs and on healthy human myotubes, with untreated cells used as controls. Results An accumulation of multivesicular bodies was observed in muscle biopsies of sporadic ALS patients by immunostaining and electron microscopy. Study of muscle biopsies and biopsy‐derived denervation‐naïve differentiated muscle stem cells (myotubes) revealed a consistent disease signature in ALS myotubes, including intracellular accumulation of exosome‐like vesicles and disruption of RNA‐processing. Compared with vesicles from healthy control myotubes, when administered to healthy MNs the vesicles of ALS myotubes induced shortened, less branched neurites, cell death, and disrupted localization of RNA and RNA‐processing proteins. The RNA‐processing protein FUS and a majority of its binding partners were present in ALS muscle vesicles, and toxicity was dependent on the expression level of FUS in recipient cells. Toxicity to recipient MNs was abolished by anti‐CD63 immuno‐blocking of vesicle uptake. Conclusions ALS muscle vesicles are shown to be toxic to MNs, which establishes the skeletal muscle as a potential source of vesicle‐mediated toxicity in ALS.

Published
2022
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21. Epigenetic immune monitoring for COVID-19 disease course prognosis

Author

Björn Samans, Marta Rosselló Chornet, Araceli Rosselló Chornet, Janine Jung, Konstantin Schildknecht, Laura Lozza, Lourdes Alos Zaragoza, Javier Hernández Laforet, Nina Babel, and Sven Olek

Subjects
COVID-19, SARS-CoV-2, epigenetic qPCR, immune monitoring, lymphopenia, disease prognosis, Immunologic diseases. Allergy, RC581-607
Abstract

BackgroundThe course of COVID-19 is associated with severe dysbalance of the immune system, causing both leukocytosis and lymphopenia. Immune cell monitoring may be a powerful tool to prognosticate disease outcome. However, SARS-CoV-2 positive subjects are isolated upon initial diagnosis, thus barring standard immune monitoring using fresh blood. This dilemma may be solved by epigenetic immune cell counting.MethodsIn this study, we used epigenetic immune cell counting by qPCR as an alternative way of quantitative immune monitoring for venous blood, capillary blood dried on filter paper (dried blood spots, DBS) and nasopharyngeal swabs, potentially allowing a home-based monitoring approach.ResultsEpigenetic immune cell counting in venous blood showed equivalence with dried blood spots and with flow cytometrically determined cell counts of venous blood in healthy subjects. In venous blood, we detected relative lymphopenia, neutrophilia, and a decreased lymphocyte-to-neutrophil ratio for COVID-19 patients (n =103) when compared with healthy donors (n = 113). Along with reported sex-related differences in survival we observed dramatically lower regulatory T cell counts in male patients. In nasopharyngeal swabs, T and B cell counts were significantly lower in patients compared to healthy subjects, mirroring the lymphopenia in blood. Naïve B cell frequency was lower in severely ill patients than in patients with milder stages.ConclusionsOverall, the analysis of immune cell counts is a strong predictor of clinical disease course and the use of epigenetic immune cell counting by qPCR may provide a tool that can be used even for home-isolated patients.

Published
2023
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22. Endothelial cells regulate astrocyte to neural progenitor cell trans-differentiation in a mouse model of stroke

Author

Li, Wenlu, Mandeville, Emiri T., Durán-Laforet, Violeta, Fukuda, Norito, Yu, Zhanyang, Zheng, Yi, Held, Aaron, Park, Ji-Hyun, Nakano, Takafumi, Tanaka, Masayoshi, Shi, Jingfei, Esposito, Elga, Niu, Wanting, Xing, Changhong, Hayakawa, Kazuhide, Lizasoain, Ignacio, van Leyen, Klaus, Ji, Xunming, Wainger, Brian J., Moro, Maria A., and Lo, Eng H.

Published
2022
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24. Understanding the content of COVID-19 vaccination and pregnancy videos on YouTube: An analysis of videos published at the start of the vaccine rollout

Author

Priscila E. Laforet, Corey H. Basch, and Hao Tang

Subjects
youtube, social media, misinformation, vaccination, pregnancy, covid-19, Immunologic diseases. Allergy, RC581-607, Therapeutics. Pharmacology, RM1-950
Abstract

Over 2 years into the COVID-19 pandemic, information on the safety and efficacy of COVID-19 vaccination, particularly for people in high-risk populations, has become a popular topic of discussion. The purpose of this study was to analyze the content and characteristics of YouTube videos related to COVID-19 vaccination and pregnancy. The 50 most viewed English language videos on pregnancy and COVID-19 vaccination were included in this study. The 50 YouTube videos were viewed 4,589,613 times, with 6% uploaded by consumers, 40% by medical professionals, and 44% by television or internet-based news. Videos from consumer sources more often mentioned a human trial of the COVID-19 vaccine (75% of consumer videos vs. 65% of medical professional videos and 31.8% of television or internet-based news videos, P = .036) and more often mentioned anti-vaccination sentiment, fear, or distrust of the vaccines (37.5% of consumer videos vs 5.0% of medical professional videos and 4.5% of television or internet-based news videos, P = .018). Videos uploaded by medical professionals more often mentioned emergency use of the COVID-19 vaccines (P = .016), passive immunity in general (P = .011), and that the COVID-19 vaccine is either unlikely to or will not cause harm in breastfeeding more often than did videos from consumer or television-based news sources (P = .034). New information regarding COVID-19 vaccination and pregnancy is continuing to emerge, and this study highlights that the information found in the most viewed YouTube videos on this topic can quickly become outdated.

Published
2022
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25. A study on the safety and efficacy of reveglucosidase alfa in patients with late-onset Pompe disease

Author

Byrne, Barry J, Geberhiwot, Tarekegn, Barshop, Bruce A, Barohn, Richard, Hughes, Derralynn, Bratkovic, Drago, Desnuelle, Claude, Laforet, Pascal, Mengel, Eugen, Roberts, Mark, Haroldsen, Peter, Reilley, Kristin, Jayaram, Kala, Yang, Ke, Walsh, Liron, and on behalf of the POM-001/002 Investigators

Subjects
Biomedical and Clinical Sciences, Clinical Sciences, Chronic Liver Disease and Cirrhosis, Rare Diseases, Liver Disease, Lung, Clinical Research, Digestive Diseases, 6.1 Pharmaceuticals, Evaluation of treatments and therapeutic interventions, Adult, Enzyme Replacement Therapy, Female, Glycogen Storage Disease Type II, Humans, Male, Middle Aged, Muscle, Skeletal, alpha-Glucosidases, Reveglucosidase alfa, Late-onset Pompe disease, Enzyme replacement therapy, Pharmacokinetics, Safety, Efficacy, Respiratory, Pulmonary, POM-001/002 Investigators, Other Medical and Health Sciences, Genetics & Heredity, Genetics, Clinical sciences
Abstract

BackgroundLate-onset Pompe disease is a rare genetic neuromuscular disorder caused by lysosomal acid alpha-glucosidase (GAA) deficiency that ultimately results in mobility loss and respiratory failure. Current enzyme replacement therapy with recombinant human (rh)GAA has demonstrated efficacy in subjects with late-onset Pompe disease. However, long-term effects of rhGAA on pulmonary function have not been observed, likely related to inefficient delivery of rhGAA to skeletal muscle lysosomes and associated deficits in the central nervous system. To address this limitation, reveglucosidase alfa, a novel insulin-like growth factor 2 (IGF2)-tagged GAA analogue with improved lysosomal uptake, was developed. This study evaluated the pharmacokinetics, safety, and exploratory efficacy of reveglucosidase alfa in 22 subjects with late-onset Pompe disease who were previously untreated with rhGAA.ResultsReveglucosidase alfa plasma concentrations increased linearly with dose, and the elimination half-life was

Published
2017

26. Duvoglustat HCl Increases Systemic and Tissue Exposure of Active Acid α-Glucosidase in Pompe Patients Co-administered with Alglucosidase α

Author

Kishnani, Priya, Tarnopolsky, Mark, Roberts, Mark, Sivakumar, Kumarswamy, Dasouki, Majed, Dimachkie, Mazen M, Finanger, Erika, Goker-Alpan, Ozlem, Guter, Karl A, Mozaffar, Tahseen, Pervaiz, Muhammad Ali, Laforet, Pascal, Levine, Todd, Adera, Matthews, Lazauskas, Richard, Sitaraman, Sheela, Khanna, Richie, Benjamin, Elfrida, Feng, Jessie, Flanagan, John J, Barth, Jay, Barlow, Carrolee, Lockhart, David J, Valenzano, Kenneth J, Boudes, Pol, Johnson, Franklin K, and Byrne, Barry

Subjects
Pharmacology and Pharmaceutical Sciences, Biomedical and Clinical Sciences, Rare Diseases, Chronic Liver Disease and Cirrhosis, Digestive Diseases, Liver Disease, 5.1 Pharmaceuticals, 6.1 Pharmaceuticals, Evaluation of treatments and therapeutic interventions, Development of treatments and therapeutic interventions, 1-Deoxynojirimycin, Administration, Oral, Adult, Drug Administration Schedule, Drug Synergism, Drug Therapy, Combination, Enzyme Replacement Therapy, Female, Glycogen Storage Disease Type II, Humans, Infusions, Intravenous, Lysosomes, Male, Middle Aged, Muscle, Skeletal, Patient Safety, Treatment Outcome, alpha-Glucosidases, Pompe disease, enzyme replacement therapy, pharmacokinetics, pharmacological chaperone, Biological Sciences, Technology, Medical and Health Sciences, Biotechnology, Genetics, Clinical sciences, Medical biotechnology
Abstract

Duvoglustat HCl (AT2220, 1-deoxynojirimycin) is an investigational pharmacological chaperone for the treatment of acid α-glucosidase (GAA) deficiency, which leads to the lysosomal storage disorder Pompe disease, which is characterized by progressive accumulation of lysosomal glycogen primarily in heart and skeletal muscles. The current standard of care is enzyme replacement therapy with recombinant human GAA (alglucosidase alfa [AA], Genzyme). Based on preclinical data, oral co-administration of duvoglustat HCl with AA increases exposure of active levels in plasma and skeletal muscles, leading to greater substrate reduction in muscle. This phase 2a study consisted of an open-label, fixed-treatment sequence that evaluated the effect of single oral doses of 50 mg, 100 mg, 250 mg, or 600 mg duvoglustat HCl on the pharmacokinetics and tissue levels of intravenously infused AA (20 mg/kg) in Pompe patients. AA alone resulted in increases in total GAA activity and protein in plasma compared to baseline. Following co-administration with duvoglustat HCl, total GAA activity and protein in plasma were further increased 1.2- to 2.8-fold compared to AA alone in all 25 Pompe patients; importantly, muscle GAA activity was increased for all co-administration treatments from day 3 biopsy specimens. No duvoglustat-related adverse events or drug-related tolerability issues were identified.

Published
2017

28. Loss of Weight Gained During the COVID-19 Pandemic: Content Analysis of YouTube Videos

Author

Hao Tang, Sungwoo Kim, Priscila E Laforet, Naa-Solo Tettey, and Corey H Basch

Subjects
Medicine
Abstract

BackgroundMany people experienced unintended weight gain during the COVID-19 pandemic, which has been discussed widely on social media. ObjectiveThis study aims to describe the content of weight loss videos on YouTube (Google LLC) during the COVID-19 pandemic. MethodsBy using the keywords weight loss during quarantine, the 100 most viewed English-language videos were identified and coded for content related to losing weight gained during the COVID-19 pandemic. ResultsIn total, 9 videos were excluded due to having non-English content or posting data before the COVID-19 pandemic. The 91 videos included in the study sample acquired 407,326 views at the time of study and were roughly 14 minutes long. A total of 48% (44/91) of the sample videos included graphic comparisons to illustrate weight change. Videos that included a graphic comparison were more likely to have content related to trigger warnings (χ21=6.05; P=.01), weight loss (χ21=13.39; P

Published
2022
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29. Neutrophil Extracellular Trap Targeting Protects Against Ischemic Damage After Fibrin-Rich Thrombotic Stroke Despite Non-Reperfusion

Author

Carolina Peña-Martínez, Violeta Durán-Laforet, Alicia García-Culebras, María Isabel Cuartero, María Ángeles Moro, and Ignacio Lizasoain

Subjects
stroke, fibrin, NETs, neuroprotection, TLR4, Immunologic diseases. Allergy, RC581-607
Abstract

Stroke is one of the most prevalent diseases worldwide caused primarily by a thrombotic vascular occlusion that leads to cell death. To date, t-PA (tissue-type plasminogen activator) is the only thrombolytic therapy approved which targets fibrin as the main component of ischemic stroke thrombi. However, due to its highly restrictive criteria, t-PA is only administrated to less than 10% of all stroke patients. Furthermore, the research in neuroprotective agents has been extensive with no translational results from medical research to clinical practice up to now. Since we first described the key role of NETs (Neutrophil Extracellular Traps) in platelet-rich thrombosis, we asked, first, whether NETs participate in fibrin-rich thrombosis and, second, if NETs modulation could prevent neurological damage after stroke. To this goal, we have used the thromboembolic in situ stroke model which produces fibrin-rich thrombotic occlusion, and the permanent occlusion of the middle cerebral artery by ligature. Our results demonstrate that NETs do not have a predominant role in fibrin-rich thrombosis and, therefore, DNase-I lacks lytic effects on fibrin-rich thrombosis. Importantly, we have also found that NETs exert a deleterious effect in the acute phase of stroke in a platelet-TLR4 dependent manner and, subsequently, that its pharmacological modulation has a neuroprotective effect. Therefore, our data strongly support that the pharmacological modulation of NETs in the acute phase of stroke, could be a promising strategy to repair the brain damage in ischemic disease, independently of the type of thrombosis involved.

Published
2022
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30. Systematic Comparison of Uremic Toxin Removal Using Different Hemodialysis Modes: A Single-Center Crossover Prospective Observational Study

Author

Ariane Duval-Sabatier, Stephane Burtey, Marion Pelletier, Manon Laforet, Laetitia Dou, Marion Sallee, Anne-Marie Lorec, Hafssa Knidiri, Floriane Darbon, Yvon Berland, and Philippe Brunet

Subjects
dialysis, hemodiafiltration, uremic toxin removal, protein bound solutes, dialysis membrane, Biology (General), QH301-705.5
Abstract

Many hypotheses could explain the mortality decrease observed using hemodiafiltration, such as reduction of intradialytic hypotension and more efficient toxin removal. We led a systematic analysis of representative uremic toxin removal with hemodialysis (HD), online postdilution hemodiafiltration (postHDF) and online predilution hemodiafiltration (preHDF), in a single-center crossover and prospective observational study. The primary outcome was the reduction ratio of uremic toxins of the three categories defined by the Eutox group. Twenty-six patients were treated by those three techniques of extra renal epuration. Mean Kt/Vurea was not different between the treatment methods. Mean reduction ratio of beta2microglobulin was significantly higher for both HDF treatments than for HD (p < 0.001). Myoglobin, kappa, and lambda free light chain reduction ratio was significantly different between the modes: 37.75 ± 11.95%, 45.31 ± 11% and 61.22 ± 10.56%/57.21 ± 12.5%, 63.53 ± 7.93%, and 68.40 ± 11.79%/29.12 ± 8.44%, 34.73 ± 9.01%, and 45.55 ± 12.31% HD, preHDF, and postHDF, respectively (p < 0.001). Mean protein-bound solutes reduction ratio was not different between the different treatments except for PCS with a higher reduction ratio during HDF treatments. Mean albumin loss was always less than 2 g. HDF improved removal of middle molecules but had no effect on indoles concentration without any difference between synthetic dialysis membranes.

Published
2023
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31. European muscle MRI study in limb girdle muscular dystrophy type R1/2A (LGMDR1/LGMD2A)

Author

Barp, Andrea, Laforet, Pascal, Bello, Luca, Tasca, Giorgio, Vissing, John, Monforte, Mauro, Ricci, Enzo, Choumert, Ariane, Stojkovic, Tanya, Malfatti, Edoardo, Pegoraro, Elena, Semplicini, Claudio, Stramare, Roberto, Scheidegger, Olivier, Haberlova, Jana, Straub, Volker, Marini-Bettolo, Chiara, Løkken, Nicoline, Diaz-Manera, Jordi, Urtizberea, Jon A., Mercuri, Eugenio, Kynčl, Martin, Walter, Maggie C., and Carlier, Robert Y.

Published
2020
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32. Disease-related determinants of quality of life 10 years after clinically isolated syndrome.

Author

Kinkel, R, Laforet, Genevieve, and You, Xiaojun

Abstract

BACKGROUND: The main clinical determinants of quality of life (QOL) 5 years after clinically isolated syndrome (CIS) are Expanded Disability Status Scale (EDSS) score and conversion to clinically definite multiple sclerosis (CDMS). The aim of this study was to determine the demographic, clinical, and magnetic resonance imaging (MRI) factors associated with QOL 10 years after CIS. METHODS: Controlled High Risk Avonex® Multiple Sclerosis Prevention Study in Ongoing Neurologic Surveillance (CHAMPIONS) 10-year patients were assessed for CDMS, EDSS score, MRI T2 activity, brain parenchymal fraction, and patient-reported QOL. Associations were evaluated using analysis of variance models. RESULTS: A second clinical event consistent with CDMS and higher EDSS scores at years 5 and 10 were associated with lower 36-item Short Form Health Status Survey (SF-36) Physical Component Summary scores at year 10 (P < .01). Patients with earlier onset of CDMS had worse patient-reported Physical Component Summary, SF-36 Mental Component Summary, fatigue, and pain scores at year 10 than patients with later or no onset of CDMS. Neither initial randomization group nor any MRI metrics assessed at baseline or during follow-up were associated with QOL at 10 years. CONCLUSIONS: These results support the development of therapies for patients with CIS that significantly reduce the risk of conversion to CDMS and the progression of physical disability to milestones as low as EDSS scores of 2.0.

Published
2015

33. Role of TLR4 in Neutrophil Dynamics and Functions: Contribution to Stroke Pathophysiology

Author

Violeta Durán-Laforet, Carolina Peña-Martínez, Alicia García-Culebras, María Isabel Cuartero, Eng H. Lo, María Ángeles Moro, and Ignacio Lizasoain

Subjects
inflammation, myeloid, stroke, neutrophil, neuroprotection, TLR4, Immunologic diseases. Allergy, RC581-607
Abstract

Background and PurposeThe immune response subsequent to an ischemic stroke is a crucial factor in its physiopathology and outcome. It is known that TLR4 is implicated in brain damage and inflammation after stroke and that TLR4 absence induces neutrophil reprogramming toward a protective phenotype in brain ischemia, but the mechanisms remain unknown. We therefore asked how the lack of TLR4 modifies neutrophil function and their contribution to the inflammatory process.MethodsIn order to assess the role of the neutrophilic TLR4 after stroke, mice that do not express TLR4 in myeloid cells (TLR4loxP/Lyz-cre) and its respective controls (TLR4loxP/loxP) were used. Focal cerebral ischemia was induced by occlusion of the middle cerebral artery and infarct size was measured by MRI. A combination of flow cytometry and confocal microscopy was used to assess different neutrophil characteristics (circadian fluctuation, cell surface markers, cell complexity) and functions (apoptosis, microglia engulfment, phagocytosis, NETosis, oxidative burst) in both genotypes.ResultsAs previously demonstrated, mice with TLR4 lacking-neutrophils had smaller infarct volumes than control mice. Our results show that the absence of TLR4 keeps neutrophils in a steady youth status that is dysregulated, at least in part, after an ischemic insult, preventing neutrophils from their normal circadian fluctuation. TLR4-lacking neutrophils showed a higher phagocytic activity in the basal state, they were preferentially engulfed by the microglia after stroke, and they produced less radical oxygen species (ROS) in the first stage of the inflammatory process.ConclusionsTLR4 is specifically involved in neutrophil dynamics under physiological conditions as well as in stroke-induced tissue damage. This research contributes to the idea that TLR4, especially when targeted in specific cell types, is a potential target for neuroprotective strategies.

Published
2021
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35. Toxoplasma gondii seroprevalence in extensively farmed wild boars (Sus scrofa) in Denmark

Author

Celine Kaae Laforet, Gunita Deksne, Heidi Huus Petersen, Pikka Jokelainen, Maria Vang Johansen, and Brian Lassen

Subjects
Serology, Sus scrofa, Toxoplasma gondii, Wild boar, Zoonosis, Veterinary medicine, SF600-1100
Abstract

Abstract Toxoplasma gondii is a zoonotic parasite of worldwide importance. In this study, we estimated T. gondii seroprevalence in extensively farmed wild boars in Denmark, where little is known about T. gondii in animal hosts. Our study focused on wild boars because they are considered good indicator species for the presence of T. gondii, and wild boar meat is used for human consumption. Serum samples from 101 wild boars collected in 2016–2018 from five different locations from the continental part of Denmark, Jutland, were screened for anti-T. gondii antibodies. The samples were analysed using a commercial indirect enzyme-linked immunosorbent assay (ELISA). Samples from 28 (27.7%) of the 101 wild boars tested positive with the ELISA. The odds for a wild boar to test seropositive were higher if it was sampled during the hunting season 2017–2018 than during 2016–2017 and if it was reported to be at least 1 year old than if it was younger (logistic regression model with the two variables: odds ratios 17.5 and 3.9, respectively). A substantial proportion of the investigated extensively farmed wild boars had been exposed to T. gondii. Moreover, the parasite appeared widespread, at least in the continental part of Denmark, Jutland, as seropositive wild boars were found from all five sampled locations. Assuming seropositivity indicates hosting viable parasites, consumption of undercooked wild boar meat from Denmark is a potential source of T. gondii infections to other hosts, including humans.

Published
2019
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36. European consensus for starting and stopping enzyme replacement therapy in adult patients with Pompe disease: a 10-year experience

Author

A. T. van der Ploeg, M. Ую Kruijshaar, A. Toscano, P. Laforet, C. Angelini, R. H. Lachmann, S. I. Pascual Pascual, M. Roberts, K. Rosler, T. Stulnig, P. A. van Doorn, P.Y. K. Van den Bergh, J. Vissing, and B. Schoser

Subjects
взрослые пациенты, алглюкозидаза альфа, ферментная заместительная терапия, руководство, болезнь помпе, рекомендации по лечению, Neurology. Diseases of the nervous system, RC346-429
Published
2019
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42. A study on the safety and efficacy of reveglucosidase alfa in patients with late-onset Pompe disease

Author

Barry J. Byrne, Tarekegn Geberhiwot, Bruce A. Barshop, Richard Barohn, Derralynn Hughes, Drago Bratkovic, Claude Desnuelle, Pascal Laforet, Eugen Mengel, Mark Roberts, Peter Haroldsen, Kristin Reilley, Kala Jayaram, Ke Yang, Liron Walsh, and on behalf of the POM-001/002 Investigators

Subjects
Reveglucosidase alfa, Late-onset Pompe disease, Enzyme replacement therapy, Pharmacokinetics, Safety, Efficacy, Medicine
Abstract

Abstract Background Late-onset Pompe disease is a rare genetic neuromuscular disorder caused by lysosomal acid alpha-glucosidase (GAA) deficiency that ultimately results in mobility loss and respiratory failure. Current enzyme replacement therapy with recombinant human (rh)GAA has demonstrated efficacy in subjects with late-onset Pompe disease. However, long-term effects of rhGAA on pulmonary function have not been observed, likely related to inefficient delivery of rhGAA to skeletal muscle lysosomes and associated deficits in the central nervous system. To address this limitation, reveglucosidase alfa, a novel insulin-like growth factor 2 (IGF2)-tagged GAA analogue with improved lysosomal uptake, was developed. This study evaluated the pharmacokinetics, safety, and exploratory efficacy of reveglucosidase alfa in 22 subjects with late-onset Pompe disease who were previously untreated with rhGAA. Results Reveglucosidase alfa plasma concentrations increased linearly with dose, and the elimination half-life was

Published
2017
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43. Delayed Effects of Acute Reperfusion on Vascular Remodeling and Late-Phase Functional Recovery After Stroke

Author

Violeta Durán-Laforet, David Fernández-López, Alicia García-Culebras, Juan González-Hijón, Ana Moraga, Sara Palma-Tortosa, Isaac García-Yébenes, Adrián Vega-Pérez, Ignacio Lizasoain, and María Ángeles Moro

Subjects
brain, recanalization, perfusion, blood-brain barrier, angiogenesis, stroke, Neurosciences. Biological psychiatry. Neuropsychiatry, RC321-571
Abstract

Tissue perfusion is a necessary condition for vessel survival that can be compromised under ischemic conditions. Following stroke, delayed effects of early brain reperfusion on the vascular substrate necessary for remodeling, perfusion and maintenance of proper peri-lesional hemodynamics are unknown. Such aspects of ischemic injury progression may be critical for neurological recovery in stroke patients. This study aims to describe the impact of early, non-thrombolytic reperfusion on the vascular brain component and its potential contribution to tissue remodeling and long-term functional recovery beyond the acute phase after stroke in 3-month-old male C57bl/6 mice. Permanent (pMCAO) and transient (60 min, tMCAO) brain ischemia mouse models were used for characterizing the effect of early, non-thrombolytic reperfusion on the brain vasculature. Analysis of different vascular parameters (vessel density, proliferation, degeneration and perfusion) revealed that, while early middle cerebral artery recanalization was not sufficient to prevent sub-acute vascular degeneration within the ischemic brain regions, brain reperfusion promoted a secondary wave of vascular remodeling in the peri-lesional regions, which led to improved perfusion of the ischemic boundaries and late-phase neurological recovery. This study concluded that acute, non-thrombolytic artery recanalization following stroke favors late-phase vascular remodeling and improves peri-lesional perfusion, contributing to secondary functional recovery.

Published
2019
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44. Diaphragm sniff ultrasound: Normal values, relationship with sniff nasal pressure and accuracy for predicting respiratory involvement in patients with neuromuscular disorders.

Author

Abdallah Fayssoil, Lee S Nguyen, Adam Ogna, Tanya Stojkovic, Paris Meng, Dominique Mompoint, Robert Carlier, Helene Prigent, Bernard Clair, Anthony Behin, Pascal Laforet, Guillaume Bassez, Pascal Crenn, David Orlikowski, Djillali Annane, Bruno Eymard, and Frederic Lofaso

Subjects
Medicine, Science
Abstract

BackgroundIn patients with neuromuscular disorders, assessment of respiratory function relies on forced vital capacity (FVC) measurements. Providing complementary respiratory outcomes may be useful for clinical trials. Diaphragm sniff ultrasound (US) is a noninvasive technique that can assess diaphragm function that may be affected in patients with neuromuscular disorders.PurposeWe aimed to provide normal values of sniff diaphragm ultrasound, to assess the relationship between sniff diaphragm US, vital capacity (VC) and sniff nasal pressure. Additionally, we aimed to evaluate the diagnostic accuracy of sniff diaphragm US for predicting restrictive pulmonary insufficiency.Materials and methodsWe included patients with neuromuscular disorders that had been tested with a sniff diaphragm US and functional respiratory tests. Healthy subjects were also included to obtain normal diaphragm sniff ultrasound. We performed diaphragm tissue Doppler imaging (TDI) and time movement (TM) diaphragm echography combined with sniff maneuver.ResultsA total of 89 patients with neuromuscular diseases and 27 healthy subjects were included in our study. In patients, the median age was 32 years [25; 50] and the median FVC was 34% of predicted [18; 55]. Sniff diaphragm motion using TM ultrasound was significantly associated with sniff nasal pressure, both for the right hemidiaphragm (r = 0.6 p ConclusionSniff diaphragm TM and TDI measures were significantly associated with sniff nasal pressure. Sniff diaphragm TM and TDI had a high level of accuracy to reveal respiratory involvement in patients with neuromuscular disorders. This technique is useful to assess and follow up diaphragm function in patients with neuromuscular disorders. It may be used as a respiratory outcome for clinical trials.

Published
2019
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46. Epidemiology of myasthenia gravis in France: A retrospective claims database study (STAMINA)

Author

Salort-Campana, E., Laforet, P., de Pouvourville, G., Crochard, A., Chollet, G., Nevoret, C., Emery, C., Bouée, S., and Tard, C.

Abstract

•We found a higher incidence and prevalence of MG are higher than previously reported in most European countries.•AChEIs are the most common treatment and more aggressive treatments (thymectomy, IVIg, azathioprine, plasma exchange, mycophenolate mofetil, cyclophosphamide, and rituximab) are mainly used the first year of the disease history.•Despite the availability of several treatments, patients with MG still experience exacerbations and crises and have an excess mortality compared to controls without MG, regardless of comorbidities.

Published
2024
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48. Affected female carriers of MTM1 mutations display a wide spectrum of clinical and pathological involvement: delineating diagnostic clues

Author

Biancalana, Valérie, Scheidecker, Sophie, Miguet, Marguerite, Laquerrière, Annie, Romero, Norma B., Stojkovic, Tanya, Abath Neto, Osorio, Mercier, Sandra, Voermans, Nicol, Tanner, Laura, Rogers, Curtis, Ollagnon-Roman, Elisabeth, Roper, Helen, Boutte, Célia, Ben-Shachar, Shay, Lornage, Xavière, Vasli, Nasim, Schaefer, Elise, Laforet, Pascal, Pouget, Jean, Moerman, Alexandre, Pasquier, Laurent, Marcorelle, Pascale, Magot, Armelle, Küsters, Benno, Streichenberger, Nathalie, Tranchant, Christine, Dondaine, Nicolas, Schneider, Raphael, Gasnier, Claire, Calmels, Nadège, Kremer, Valérie, Nguyen, Karine, Perrier, Julie, Kamsteeg, Erik Jan, Carlier, Pierre, Carlier, Robert-Yves, Thompson, Julie, Boland, Anne, Deleuze, Jean-François, Fardeau, Michel, Zanoteli, Edmar, Eymard, Bruno, and Laporte, Jocelyn

Published
2017
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49. Proceedings of Réanimation 2017, the French Intensive Care Society International Congress

Author

Bougouin, Wulfran, Marijon, Eloi, Planquette, Benjamin, Karam, Nicole, Dumas, Florence, Celermajer, David, Jost, Daniel, Lamhaut, Lionel, Beganton, Frankie, Cariou, Alain, Meyer, Guy, Jouven, Xavier, Bureau, Côme, Charpentier, Julien, Salem, Omar Ben Hadj, Guillemet, Lucie, Arnaout, Michel, Ferre, Alexis, Geri, Guillaume, Mongardon, Nicolas, Pène, Frédéric, Chiche, Jean-Daniel, Mira, Jean-Paul, Labro, Guylaine, Belon, François, Luu, Vinh-Phuc, Chenet, Julien, Besch, Guillaume, Puyraveau, Marc, Piton, Gaël, Capellier, Gilles, Martin, Maëlle, Lascarrou, Jean-Baptiste, Le Thuaut, Aurélie, Lacherade, Jean-Claude, Martin-Lefèvre, Laurent, Fiancette, Maud, Vinatier, Isabelle, Lebert, Christine, Bachoumas, Konstantinos, Yehia, Aihem, Henry-Laguarrigue, Matthieu, Colin, Gwenhaël, Reignier, Jean, Privat, Elodie, Escutnaire, Joséphine, Dumont, Cyrielle, Baert, Valentine, Vilhelm, Christian, Hubert, Hervé, Robert-Edan, Vincent, Lakhal, Karim, Quartin, Andrew, Hobbs, Brian, Cely, Cynthia, Bell, Cynthia, Pham, Tai, Schein, Roland, Geng, Yimin, Ng, Chaan, Ehrmann, Stephan, Gandonnière, Charlotte Salmon, Boisramé-Helms, Julie, Le Tilly, Olivier, De Bretagne, Isabelle Benz, Mercier, Emmanuelle, Mankikian, Julie, Bretagnol, Anne, Meziani, Ferhat, Halimi, Jean Michel, Le Guellec, Chantal Barin, Gaudry, Stéphane, Hajage, David, Tubach, Florence, Pons, Bertrand, Boulet, Eric, Boyer, Alexandre, Chevrel, Guillaume, Lerolle, Nicolas, Carpentier, Dorothée, de Prost, Nicolas, Lautrette, Alexandre, Mayaux, Julien, Nseir, Saad, Ricard, Jean-Damien, Dreyfuss, Didier, Robert, René, Garzotto, Franscesco, Kipnis, Eric, Tetta, Ciro, Ronco, Claudio, Schnell, David, Aurelie, Bourmaud, Reynaud, Marie, Clec’h, Christophe, Benyamina, Mourad, Vincent, François, Mariat, Christophe, Bornstain, Caroline, Rouleau, Stephane, Leroy, Christophe, Cohen, Yves, Morel, Jerome, Legrand, Matthieu, Terreaux, Jeremy, Darmon, Michaël, Cantier, Marie, Morisot, Adeline, Guérot, Emmanuel, Canet, Emmanuel, De Montmollin, Etienne, Voiriot, Guillaume, Neuville, Mathilde, Timsit, Jean-François, Sonneville, Romain, Fayssoil, Abdallah, Stojkovic, Tania, Behin, Anthony, Ogna, Adam, Lofaso, Frédéric, Laforet, Pascal, Wahbi, Karim, Prigent, Helene, Duboc, Denis, Orlikowski, David, Eymard, Bruno, Annane, Djillali, Le Guennec, Loic, Cholet, Clémentine, Bréchot, Nicolas, Hekimian, Guillaume, Besset, Sébastien, Lebreton, Guillaume, Nieszkowska, Ania, Trouillet, Jean Louis, Leprince, Pascal, Combes, Alain, Luyt, Charles-Edouard, Griton, Marion, Sesay, Musa, De Panthou, Nadia Sibaï, Bienvenu, Thomas, Biais, Matthieu, Nouette-Gaulain, Karine, Fossat, Guillaume, Baudin, Florian, Coulanges, Cécile, Bobet, Sabrine, Dupont, Arnaud, Courtes, Léa, Benzekri, Dalila, Kamel, Toufik, Muller, Grégoire, Bercault, Nicolas, Barbier, François, Runge, Isabelle, Skarzynski, Marie, Mathonnet, Armelle, Boulain, Thierry, Jouan, Youenn, Teixera, Noémie, Hassen-Khodja, Claire, Guillon, Antoine, Gaborit, Christophe, Grammatico-Guillon, Leslie, Rebière, Cécile, Azoulay, Elie, Misset, Benoit, Ruckly, Stephane, Garrouste-Orgeas, Maïté, Kentish-Barnes, Nancy, Duranteau, Jacques, Thuong, Marie, Joseph, Liliane, Renault, Anne, Lesieur, Olivier, Larbi, Anne-Gaelle Si, Viquesnel, Gérald, Zuber, Benjamin, Marque, Sophie, Kandelman, Stanislas, Pichon, Nicolas, Floccard, Bernard, Galon, Marion, Chevret, Sylvie, Kentish-Barnes, Nancy, Seegers, Valérie, Legriel, Stéphane, Jaber, Samir, Lefrant, Jean Yves, Reuter, Danielle, Guisset, Olivier, Cracco, Christophe, Seguin, Amélie, Durand-Gasselin, Jacques, Thirion, Marine, Cohen-Solal, Zoé, Foulgoc, Hélène, Rogier, Julien, Delobbe, Elsa, Schortgen, Frédérique, Asfar, Pierre, Julie, Boisramé-Helms, Grimaldi, David, Fabien, Grelon, Anguel, Nadia, Sigismond, Lasocki, Matthieu, Henry-Lagarrigue, Gonzalez, Frédéric, François, Legay, Guitton, Christophe, Schenck, Maleka, Jean-Marc, Doise, Radermacher, Peter, Kentish-Barnes, Nancy, Makunza, Joseph Nsiala, Nathalie, Mejeni Kamdem, Pierre, Akilimali, Adolphe, Kilembe Manzanza, Mahieu, Rafael, Reydel, Thomas, Jamet, Angéline, Chudeau, Nicolas, Huntzinger, Julien, Grange, Steven, Courte, Anne, Lemarie, Jérémie, Gibot, Sébastien, Champey, Julia, Dellamonica, Jean, Du Cheyron, Damien, Contou, Damien, Tadié, Jean-Marc, Cour, Martin, Beduneau, Gaetan, Marchalot, Antoine, Guérin, Laurent, Jochmans, Sebastien, Terzi, Nicolas, Preau, Sebastien, Brun-Buisson, Christian, Dessap, Armand Mekontso, Vedrenne-Cloquet, Meryl, Breinig, Sophie, Jung, Camille, Brussieux, Maxime, Marcoux, Marie-Odile, Durrmeyer, Xavier, Blondé, Renaud, Angoulvant, François, Grasset, Jérôme, Naudin, Jérôme, Dauger, Stéphane, Remy, Solenn, Kolev-Descamp, Karine, Demaret, Julie, Monneret, Guillaume, Javouhey, Etienne, Chomton, Maryline, Sauthier, Michaël, Vallieres, Emilie, Jouvet, Philippe, Geslain, Guillaume, Guellec, Isabelle, Rambaud, Jérôme, Schmidt, Matthieu, Schellongowski, Peter, Dorget, Amandine, Patroniti, Nicolo, Taccone, Fabio Silvio, Miranda, Dinis 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K., Chlilek, A., Hajji, Ahmed, Louati, Assaad, Khaldi, Ammar, Borgi, Aida, Ghali, Nargess, Bouziri, Asma, Menif, Khaled, Ben, Jaballah Najla, Armel, Anwar, Brochon, Jeanne, Dumitrescu, Mihaela, Thévenot, Sarah, Saulnier, Jean-Pascal, Husseini, Khaled, Laland, Catherine, Cremniter, Julie, Bousseau, Anne, Castel, Olivier, Brémaud-Csizmadia, Cassandra, Diss, Margot, Portefaix, Aurélie, Berthiller, Julien, Gillet, Yves, Aoul, Nabil Tabet, Douah, Ali, Addou, Zakaria, Youbi, Houari, Moussati, Mohamed, Belhabiche, Kamel, Mir, Souad, Abada, Sanaa, Amel, Zerhouni, Aouffen, Nabil, Bouzit, Zina, Grati, Ahmed H., Dhonneur, Gilles F., Boussarsar, Mohamed, Lau, Nicolas, Mezhari, Ilham, Roucaud, Nicolas, Le Meur, Matthieu, Paulet, Rémi, Coudray, Jean-Michel, Ghomari, Wahiba Imène, Boumlik, Reda, Peigne, Vincent, Daban, Jean-Louis, Boutonnet, Mathieu, Lenoir, Bernard, Yassine, Hafiani, Mohamed, Cheikh Chaigar, Khalid, Allali, Ihssan, Moussaid, Said, Elyoussoufi, Said, Salmi, Jazia, Amira Ben, Fatima, 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Andrejak, Claire, Ricome, Sylvie, Dupont, Hervé, Baudin, François, Dureau, Pauline, Tanguy, Audrey, Arbelot, Charlotte, Ben, Hassen Kais, Charfeddine, Ahmed, Granger, Benjamin, Laporte, Lucile, Hermetet, Coralie, Regaieg, Kais, Khemakhem, Rim, Chelly, Hedi, Cheikh, Chaigar Mohammed, Mountij, Hamid, Rghioui, Kawtar, Haddad, Wafae, Cherkab, Rachid, Barrou, Houcine, Naima, Aitmouden, bennani, Othmani M., Regaieg, Kais, Douib, Ahmed, Samet, Amal, Cungi, Pierre-Julien, Nguyen, Cédric, Cotte, Jean, D’aranda, Erwan, Meaudre, Eric, Avaro, Jean-Phillipe, Slaoui, Mohamed Taoufik, Mokline, Amel, Rahmani, Imene, Laajili, Achraf, Amri, Helmi, Gharsallah, Lazheri, Gasri, Bahija, Tlaili, Sofiene, Hammouda, Rym, Messadi, Amen Allah, Sudden Death Expertise Center, AKIKI Study Group, DO-RE-MI-FA Group, ENCEPHALITICA Study Group, for the HYPER2S Investigators and REVA Research Network, for the Purpura Fulminans Study Group, GFRUP RMEF, REVA ECMOnet, REA-RAISIN Study Group, for the EurêClark Study Group, and Groupe Communication et Simulation en Pédiatrie

Published
2017
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50. Left bundle branch block in Duchenne muscular dystrophy: Prevalence, genetic relationship and prognosis.

Author

Abdallah Fayssoil, Rabah Ben Yaou, Adam Ogna, Cendrine Chaffaut, France Leturcq, Olivier Nardi, Karim Wahbi, Denis Duboc, Frederic Lofaso, Helene Prigent, Bernard Clair, Pascal Crenn, Guillaume Nicolas, Pascal Laforet, Anthony Behin, Sylvie Chevret, David Orlikowski, and Djillali Annane

Subjects
Medicine, Science
Abstract

Duchenne muscular dystrophy (DMD) is an inherited myogenic disorder due to mutations in the dystrophin gene on chromosome Xp21.1. We designed this study to determine the prevalence of left bundle branch block (LBBB), whether there is a relationship between LBBB and genetic pattern, and to assess predictive factors for acute cardiac events and mortality in adult DMD patients.We reviewed the charts of DMD followed at the Home Mechanical Ventilation Unit of the Raymond Poincare University Hospital.A total of 121 patients, aged from 18 to 41 years have been included in our study. Median vital capacity (VC) was 12% [7; 19.5] of predicted. Almost all patients were on home mechanical ventilation (95%). LBBB was present in 15 patients (13%); among them, 10 disclosed exonic deletions. After a median follow up of 6 years, 21 patients (17%) experienced acute heart failure (AHF), 7 patients (6%) supraventricular arrhythmia, 3 patients (2.4%) ventricular tachycardia, 4 patients (3%) significant electrical disturbances. LBBB was significantly associated with cardiac events (OR = 12.7; 95%CI [3.78-42.7]; p

Published
2018
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