Results 91 to 100 of about 110,785 (312)
PolyG Fibrils Coalesce Into Nuclear Ribbons That Engage Proteostasis Machinery in Neuronal Intranuclear Inclusion Disease
Advanced Science, EarlyView.In NIID, expanded NOTCH2NLC repeats give rise to nuclear polyG inclusions. Tracer‐guided in situ cryo‐electron tomography enables cross‐scale structural analysis from mouse brain to native neuronal nuclei, revealing dense‐core/peripheral‐halo inclusions built from compact polyG ribbons.Hui Dong, Yongcheng Pan, Zhiyao Tang, Yuxuan Yao, Guicong Zhang, Junpu Wang, Haonan Xiao, Yun Tian, Beisha Tang, Dan Li, Qiang Guo, Ruijun Tian, Qiong Liu, Cong Liu +13 morewiley +1 more sourceClinical implications of blood eosinophil count in patients with non-asthma–COPD overlap syndrome COPD [PDF]
, 2017 Jin Hwa Song,1 Chang-Hoon Lee,1 Jin Woo Kim,2 Won-Yeon Lee,3 Ji Ye Jung,4 Joo Hun Park,5 Ki Suck Jung,6 Kwang Ha Yoo,7 Yong Bum Park,8 Deog Keom Kim9 1Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Seoul National ...Lee C, Park JH, Song JH, Jung KS, Lee WY, Jung JY, Yoo KH, Kim JW, Kim DK, Park YB +9 morecore Revisiting the NIH Taskforce on the Research needs of Eosinophil-Associated Diseases (RE-TREAD) [PDF]
, 2018 Eosinophil-associated diseases (EADs) are rare, heterogeneous disorders characterized by the presence of eosinophils in tissues and/or peripheral blood resulting in immunopathology.Collins, Margaret M.H., Joseph R Arron, PF Weller (7781321), Khoury, Paneez, Leiferman, Kristen M, KM Leiferman (7781285), Steven J Ackerman, Spencer, Lisa A, P Khoury (7781252), Gerald J Gleich, SJ Ackerman (7781258), Akuthota, Praveen, M Minnicozzi (7781294), Steinfeld, Jonathan, Fulkerson, Patricia C, Fulkerson, Patricia P.C., Minnicozzi, Michael, Leiferman, Kristin, Jean-Emmanuel Kahn, Gopal-Srivastava, Rashmi, Gleich, Gerald J, Kahn, Jean-Emmanuel, JR Arron (7781261), Sameer K Mathur, SK Mathur (7781291), Arron, Joseph R, LA Spencer (7781312), Wechsler, Michael M.E., AJ Wardlaw (7654397), J Steinfeld (7781315), Arron, Joseph J.R., R Gopal-Srivastava (7781279), Ackerman, Steven J, Bruce S Bochner, Elizabeth A Jacobsen, GJ Gleich (7781276), PC Fulkerson (7781273), Prussin, Calman, J-E Kahn (7781270), C Prussin (7781297), Spencer, Lisa L.A., Weller, Peter, Peter F Weller, ME Wechsler (7781318), Sable, Kathleen, Wardlaw, Andrew J, Hans-Uwe Simon, Wechsler, Michael E, Amy D Klion, Francesca, Levi-Schaffer, Kathleen Sable, Rothenberg, Marc, Francesca, Levi Schaffer, P Akuthota (7781255), Ackerman, Steven S.J., Bochner, Bruce B.S., Bochner, Bruce S, Paneez Khoury, D Simon (7781309), Weller, Peter F, Wardlaw, Andrew, Patricia C Fulkerson, MH Collins (7781267), Jacobsen, Elizabeth A, Rothenberg, Marc E, Jonathan Steinfeld, Roufosse, Florence, Michael Minnicozzi, Mathur, Sameer S.K., ME Rothenberg (7781300), Michael E Wechsler, Florence Roufosse, EA Jacobsen (7781282), Marc E Rothenberg, Jacobsen, Elizabeth E.A., Margaret H Collins, L-S Francesca (7781288), AD Klion (7781324), Praveen Akuthota, Kristen M Leiferman, Lisa A Spencer, Dagmar Simon, Andrew J Wardlaw, K Sable (7781306), Klion, Amy D, Calman Prussin, H-U Simon (7737986), BS Bochner (7781264), Gleich, Gerald G.J., Simon, Hans-Uwe, Klion, Amy, Rashmi Gopal-Srivastava, F Roufosse (7781303), Mathur, Sameer K, Simon, Dagmar, Collins, Margaret H, Levi-Schaffer Francesca +96 morecore +1 more sourceA Second Pathogenic Protein, PolyGN2C‐iso2, Reveals a Dual‐Protein Pathology in Neuronal Intranuclear Inclusion Disease
Advanced Science, EarlyView.This study reveals that NOTCH2NLC transcript variant 2 generates PolyGN2C‐iso2, an aggregating protein present within intranuclear inclusions of NIID patient tissues. A novel mouse model expressing PolyGN2C‐iso2 recapitulates white matter abnormalities and cognitive deficits, mechanistically linked to mitochondrial dysfunction. These findings support a Kang Zhang, Wenhao Ma, Yi Zhou, Zhijie Wu, Pan Gao, Hongze Niu, Hongfei Tai, Tianyi Zhao, Zheyue Dong, Li Li, Yan Zhang, An Wang, Si Shen, Yueyang Li, Sifei Yu, Yan Peng, Wang Sheng, Xiaoyan Dong, Hua Pan, Kaibin Shi, Magdalena J. Koziol, Xiaobing Wu, Zaiqiang Zhang +22 morewiley +1 more source[Evaluation of tissue eosinophils in eosinophilic pneumonia].
Nihon Kyobu Shikkan Gakkai zasshi, 1992 Transbronchial biopsy specimens from ten patients with eosinophilic pneumonia were analysed to see whether the tissue contained activated eosinophils or secreted eosinophil cationic protein. The specimens showed many infiltrated tissue eosinophils in the intraalveolar spaces, and thickened alveolar septae and perivascular areas.Hamada, Kaoru, Nishikawa, Kiyoshi, Ako, Hirofumi, Morii, Takeshi, Kunimatsu, Mikikazu, Cho, Sumito, Tokuyama, Takeshi, Narita, Nobuhiro +7 moreopenaire +2 more sourcesClinical Significance of PD‐L1 and HLA Expression in Esophageal Squamous Cell Carcinoma in Response to Immunotherapy
Annals of Gastroenterological Surgery, EarlyView.This study evaluated immune‐related biomarkers in esophageal squamous cell carcinoma treated with immune checkpoint inhibitors after postoperative recurrence. PD‐L1 expression in tumor‐associated macrophages and tumor cells, together with HLA class I downregulation and HLA‐DR expression, were associated with favorable responses to immunotherapy.Kosuke Kanemitsu, Yoshiyuki Tagayasu, Yoshihiro Komohara, Cheng Pan, Rin Yamada, Yukio Fujiwara, Kazuto Harada, Keisuke Kosumi, Yoshifumi Baba, Masaaki Iwatsuki +9 morewiley +1 more sourceGreater eosinophil counts at first COPD hospitalization are associated with more readmissions and fewer deaths [PDF]
, 2019 Qing Li,1 Pierre Larivée,2,3 Josiane Courteau,2 Simon Couillard,2,3 Thomas G Poder,2,4,5 Nathalie Carrier,2 Maryse Bélanger,2,3 Alain Vanasse2,5 1Center for Innovation Management Research of Xinjiang, School of Economics and Management ...Vanasse A, Larivée P, Carrier N, Poder TG, Li Q, Courteau J, Couillard S, Bélanger M +7 morecore Unraveling a Diagnostic Enigma: A TECPR2 Case Solved Through Multi‐Omic Genomics
American Journal of Medical Genetics Part A, EarlyView.ABSTRACT
TECPR2 is a key regulator of autophagy, encoded by the TECPR2 gene. Pathogenic variants in this gene have been linked to a rare hereditary sensory and autonomic neuropathy with intellectual disability (HSAN9). We report a teenage female with a syndromic intellectual disability disorder associated with neuromuscular abnormalities.Teresa Zhao, Andrew P. Fennell, Tanavi Sharma, Katrina M. Bell, Monique Dunstan, Sebastian Lunke, Meagan J. McGrath, Catriona McLean, Undiagnosed Diseases Network (UDN‐Aus), Alison Yeung, Anna Hackett, Anne Baxter, Ansley Morrish, Ashil Davawala, Azure Hermes, Ben Kamien, Ben Lundie, Carolyn Ellaway, Carolyn Shalhoub, Cas Simons, Cassandra Gray, Cathryn Poulton, Chloe Cunningham, Chris Barnett, Chris Richmond, Christopher Richards, Daniel MacArthur, Daniel Pavlic, Daniella Hock, Daniz Kooshavar, David Amor, David Mowat, Edward Formaini, Elaine Zhang, Ella Wilkins, Ella Zurita, Ellenore Martin, Elly Lynch, Emma Krzesinski, Emma Palmer, Esther Pierini, Evanthia O. Madelli, Francisco Santos Gonzalez, Gareth Baynam, Gunjan Garg, Hamish Scott, Hannah Thomson, Himanshu Goel, Ilias Goranitis, Ira Deveson, Isabella Pfundt, Jacqui Russell, Janine Smith, Jason Pinner, Julia Broadbent, Julie McGaughran, Karin Kassahn, Katherine Lewis, Kaustuv Bhattacharya, Kirsten Boggs, Kirsty West, Kristi Jones, Laura Wedd, Lauren Dreyer, Leah Frajman, Leanne Baxter, Lilian Downie, Lily Loughman, Lisa Bristowe, Lisa Ewans, Louise Cilento, Lucy Kevin, Lyndal Douglas, Madeleine Harris, Maie Walsh, Manisha Chauhan, Margit Shah, Martin Delatycki, Mathew Wallis, Matthew Hunter, Megan Ball, Megan Higgins, Meutia Kumaheri, Michael Fahey, Mike Field, Mohammadreza Hajjari, Natalie Stewart, Natalie Tan, Natasha Brown, Nicole Van Bergen, Noelia Nunez‐Martinez, Oliver Heath, Rachel Austin, Rani Sachdev, Rebecca Macintosh, Rebecca Vink, Rocio Rius, Ruvi Samarasekera, Ryan Pysar, Sandra Cooper, Sarah Casauria, Sarah Collinson, Sarah Jelenich, Sarah Josephi‐Taylor, Sarah Sandaradura, Sean Massey, Shannon Leblanc, Shuxiang Goh, Simon Bodek, Simon Sadedin, Simran Kaur, Smitha Kumble, Sue White, Suzanne Sallevelt, Tegan Stait, Tiffany Boughtwood, Tim Sikora, Timo Lassmann, Tiong Tan, Tracy Dudding, David R. Thorburn, David A. Stroud, John Christodoulou +122 morewiley +1 more source