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JOURNAL ARTICLE
Hana Smajlović, Asja Prohić
For over two decades, the acronym PAPA syndrome has been used to describe an autoinflammatory condition caused by missense mutations in the PSTPIP1 (proline-serine-threonine phosphatase interacting protein 1) gene and clinically characterized by the presence of pyogenic arthritis, pyoderma gangrenosum (PG), and acne (1,2). Due to the involvement of the PSTPIP1 gene in the regulation of innate immunity, mutations of this gene cause abnormal activation of inflammasomes, complexes of NLRP3/ASC/caspase-1 proteins...
November 2023: Acta Dermatovenerologica Croatica: ADC
#22
Maxim Yankelevich, Archana Thakur, Shakeel Modak, Roland Chu, Jeffrey Taub, Alissa Martin, Dana L Schalk, Amy Schienshang, Sara Whitaker, Katie Rea, Daniel W Lee, Qin Liu, Anthony Shields, Nai-Kong Cheung, Lawrence G Lum
Background : Since treatment of neuroblastoma (NB) with anti-GD2 monoclonal antibodies provides a survival benefit in children with minimal residual disease and our preclinical study shows that anti-CD3 x anti-GD2 bispecific antibody (GD2Bi) armed T cells (GD2BATs) were highly cytotoxic to GD2+ cell lines, we conducted a phase I/II study in recurrent/refractory patients to establish safety and explore the clinical benefit of GD2BATs. Methods: The 3+3 dose escalation study (NCT02173093) phase I involved 9 evaluable patients with NB (n=5), osteosarcoma (OST) (n=3), and desmoplastic small round cell tumors (DSRCT) (n=1) with twice weekly infusions of GD2BATs at 40, 80, or 160 x 10 6 GD2BATs/kg/infusion with daily interleukin 2 (300,000 IU/m 2 ) and twice weekly granulocyte-macrophage colony stimulating factor (250 µg/m 2 )...
November 9, 2023: Research Square
#23
JOURNAL ARTICLE
Nasikarn Angkasekiwinai, Yupin Suputtamongkol, Wiwit Tantibhedhyangkul, Nattawat Onlamoon, Pakpoom Phoompoung, Manop Pithukpakorn, Ekkapun Karuphong, Pawana Pusuwan, Pornpimon Angkasekiwinai
BACKGROUND: There is currently no effective treatment for adult-onset immunodeficiency (AOID) syndrome with anti-interferon-gamma autoantibodies (anti-IFN-γ-auto-Abs). The purpose of this study is to investigate the effectiveness of bortezomib (BTZ) for decreasing anti-IFN-γ-auto-Abs.. METHODS: A pre-and post-intervention study was conducted during February 2017-June 2019 at Siriraj Hospital (Bangkok, Thailand). Five patients were invited to receive once weekly BTZ (1...
November 8, 2023: Clinical Infectious Diseases
#24
REVIEW
Rolf Marschalek
Since the end of 2019, the SARS-CoV-2 virus started to spread in different countries, leading to a world-wide pandemia, with today's infection numbers of more than 690 million and with a case fatality rate of more than 6.9 million. In addition, about 65 million patients suffer from post/long-Covid syndromes after having infections with the SARS-CoV-2 virus or variants thereof. This review highlights the biology of the virus, summarizes our knowledge of some of the viral mechanisms that counteract our immune responses, and finally also discusses the different vaccines and their specific safety profiles...
October 31, 2023: Frontiers in Bioscience (Landmark Edition)
#25
JOURNAL ARTICLE
Priyanka Mehta, Partha Chattopadhyay, Ramakant Mohite, Ranit D'Rozario, Purbita Bandopadhyay, Jafar Sarif, Yogiraj Ray, Dipyaman Ganguly, Rajesh Pandey
Understanding the dynamic changes in gene expression during Acute Respiratory Distress Syndrome (ARDS) progression in post-acute infection patients is crucial for unraveling the underlying mechanisms. Study investigates the longitudinal changes in gene/transcript expression patterns in hospital-admitted severe COVID-19 patients with ARDS post-acute SARS-CoV-2 infection. Blood samples were collected at three time points and patients were stratified into severe and mild ARDS, based on their oxygenation saturation (SpO2 /FiO2 ) kinetics over 7 d...
January 2024: Life Science Alliance
#26
JOURNAL ARTICLE
Adam Klocperk, Marketa Bloomfield, Zuzana Parackova, Ludovic Aillot, Jiri Fremuth, Lumir Sasek, Jan David, Filip Fencl, Aneta Skotnicova, Katerina Rejlova, Martin Magner, Ondrej Hrusak, Anna Sediva
BACKGROUND: Multisystem inflammatory syndrome in children associated with COVID-19 (MIS-C) is a late complication of pediatric COVID-19, which follows weeks after the original SARS-CoV-2 infection, regardless of its severity. It is characterized by hyperinflammation, neutrophilia, lymphopenia, and activation of T cells with elevated IFN-γ. Observing the production of autoantibodies and parallels with systemic autoimmune disorders, such as systemic lupus erythematodes (SLE), we explored B cell phenotype and serum levels of type I, II, and III interferons, as well as the cytokines BAFF and APRIL in a cohort of MIS-C patients and healthy children after COVID-19...
October 28, 2023: Molecular and Cellular Pediatrics
#27
JOURNAL ARTICLE
Andrea C Wong, Ashwarya S Devason, Iboro C Umana, Timothy O Cox, Lenka Dohnalová, Lev Litichevskiy, Jonathan Perla, Patrick Lundgren, Zienab Etwebi, Luke T Izzo, Jihee Kim, Monika Tetlak, Hélène C Descamps, Simone L Park, Stephen Wisser, Aaron D McKnight, Ryan D Pardy, Junwon Kim, Niklas Blank, Shaan Patel, Katharina Thum, Sydney Mason, Jean-Christophe Beltra, Michaël F Michieletto, Shin Foong Ngiow, Brittany M Miller, Megan J Liou, Bhoomi Madhu, Oxana Dmitrieva-Posocco, Alex S Huber, Peter Hewins, Christopher Petucci, Candice P Chu, Gwen Baraniecki-Zwil, Leila B Giron, Amy E Baxter, Allison R Greenplate, Charlotte Kearns, Kathleen Montone, Leslie A Litzky, Michael Feldman, Jorge Henao-Mejia, Boris Striepen, Holly Ramage, Kellie A Jurado, Kathryn E Wellen, Una O'Doherty, Mohamed Abdel-Mohsen, Alan L Landay, Ali Keshavarzian, Timothy J Henrich, Steven G Deeks, Michael J Peluso, Nuala J Meyer, E John Wherry, Benjamin A Abramoff, Sara Cherry, Christoph A Thaiss, Maayan Levy
Post-acute sequelae of COVID-19 (PASC, "Long COVID") pose a significant global health challenge. The pathophysiology is unknown, and no effective treatments have been found to date. Several hypotheses have been formulated to explain the etiology of PASC, including viral persistence, chronic inflammation, hypercoagulability, and autonomic dysfunction. Here, we propose a mechanism that links all four hypotheses in a single pathway and provides actionable insights for therapeutic interventions. We find that PASC are associated with serotonin reduction...
October 9, 2023: Cell
#28
REVIEW
M Jumagaliyeva, D Ayaganov, S Saparbayev, N Tuychibaeva, I A Abdelazim, Y Kurmambayev, Z Khamidullina, S Yessenamanova
The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) that caused the 2019 Coronavirus Disease (COVID-19) has been associated with several neurological symptoms. This review aimed to highlight the possible mechanism of central nervous system (CNS) targeting and the neurological symptoms that may occur with the COVID-19 infection. The case reports containing the keywords "neurological symptoms" and "COVID-19" were thoroughly reviewed to identify possible mechanisms of CNS targeting and neurological manifestations associated with COVID-19 infection...
October 2023: European Review for Medical and Pharmacological Sciences
#29
JOURNAL ARTICLE
Mahmoud M Bahgat, Rola Nadeem, Mohamed H Nasraa, Khaled Amer, Wael A Hassan, Fadya M ELGarhy, Salem Reda, Dina N Abd-Elshafy
Severe acute respiratory syndrome Coronavirus 2 (SARS-CoV-2) infection is associated with proinflammatory cytokine release as mediators of host antiviral response to the infection. Cytokine persistent elevation leads to post-Coronavirus disease-2019 (COVID-19) post-COVID-19 sequela (PCS) reported in about 60% of patients affecting individual's normal life after recovery. This study evaluates relationship of cytokines and chemokines pattern during and postinfection to PCS events. Serum samples collected from 82 individuals with symptomatic, asymptomatic, or no SARS-CoV-2 infection were classified as recently or formerly infected groups according to levels of anti-2019nCoV Immunoglobulin G/Immunoglobulin M...
October 13, 2023: Viral Immunology
#30
JOURNAL ARTICLE
Menglu Yang, Vanessa Delcroix, Anton Lennikov, Nicholas Wang, Helen P Makarenkova, Darlene A Dartt
PURPOSE: Primary Sjögren's syndrome (pSS) is an autoimmune disease that mainly attacks the lacrimal glands causing severe aqueous-deficient dry eye. Clinical evidence indicates the DNA sensing mechanism in the pathogenesis of pSS. The purpose of the present study is to determine the pro-inflammatory effect of self-genomic DNA (gDNA) on myoepithelial cells (MECs), which along with acinar and ductal cells is a major cell type of the lacrimal gland. METHOD: MECs primary culture was acquired from female C57BL6J mice...
September 26, 2023: Ocular Surface
#31
JOURNAL ARTICLE
Priya Nayak, Kavitha Mukund, Shankar Subramaniam
INTRODUCTION: B cells play an integral role in the immune response to both dengue fever and COVID-19. Prior scRNAseq analyses of peripheral plasmablasts in COVID-19 have revealed a heterogeneous population with distinct cell subsets associated with proliferation; prior studies in patients with dengue fever have likewise shown the presence of proliferative pre-plasmablasts in the circulation. These findings may have implications for disease severity. In this study, we sought to gain a mechanistic understanding of the intracellular processes in naive and memory B cells that are associated with and may lead to an expanded proliferative plasmablast population in the circulation...
2023: Frontiers in Immunology
#32
JOURNAL ARTICLE
M Bello-Perez, J Hurtado-Tamayo, A Z Mykytyn, M M Lamers, R Requena-Platek, D Schipper, D Muñoz-Santos, J Ripoll-Gómez, A Esteban, P J Sánchez-Cordón, L Enjuanes, B L Haagmans, I Sola
Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) encodes six accessory proteins (3a, 6, 7a, 7b, 8, and 9b) for which limited information is available on their role in pathogenesis. We showed that the deletion of open reading frames (ORFs) 6, 7a, or 7b individually did not significantly impact viral pathogenicity in humanized K18-hACE2 transgenic mice. In contrast, the deletion of ORF8 partially attenuated SARS-CoV-2, resulting in reduced lung pathology and 40% less mortality, indicating that ORF8 is a critical determinant of SARS-CoV-2 pathogenesis...
August 25, 2023: MBio
#33
JOURNAL ARTICLE
Chiara Aloise, Jelle G Schipper, Arno van Vliet, Judith Oymans, Tim Donselaar, Daniel L Hurdiss, Raoul J de Groot, Frank J M van Kuppeveld
The nucleocapsid protein N of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) enwraps and condenses the viral genome for packaging but is also an antagonist of the innate antiviral defense. It suppresses the integrated stress response (ISR), purportedly by interacting with stress granule (SG) assembly factors G3BP1 and 2, and inhibits type I interferon responses. To elucidate its mode of action, we systematically deleted and over-expressed distinct regions and domains. We show that N via domain N2b blocks PKR-mediated ISR activation, as measured by suppression of ISR-induced translational arrest and SG formation...
August 22, 2023: PLoS Pathogens
#34
JOURNAL ARTICLE
Zesheng An, Gabriel Figueroa-Parra, Xian Zhou, Yanfeng Li, Jane Jaquith, Kathleen McCarthy-Fruin, Jennifer Sletten, Kenneth J Warrington, Cornelia Weyand, Cynthia S Crowson, Saranya Chumsri, Keith L Knutson, Alain Sanchez-Rodriguez, Uma Thanarajasingam, Alí Duarte-García, Hu Zeng
INTRODUCTION: The longitudinal responses towards multiple doses of COVID-19 mRNA vaccines in patients with systemic autoimmune diseases remain incompletely understood. While observational studies suggested the safety of COVID-19 mRNA vaccines in rheumatic disease patients, laboratory evidence is lacking. METHODS: Here we evaluated seroreactivity, clinical manifestions, and multiple disease biomarkers after 2 or 3 doses of COVID-19 mRNA vaccines in a cohort of patients with rheumatic diseases...
2023: Frontiers in Immunology
#35
JOURNAL ARTICLE
Said Assou, Engi Ahmed, Lisa Morichon, Amel Nasri, Florent Foisset, Carine Bourdais, Nathalie Gros, Sonia Tieo, Aurelie Petit, Isabelle Vachier, Delphine Muriaux, Arnaud Bourdin, John De Vos
Airway-liquid interface cultures of primary epithelial cells and of induced pluripotent stem-cell-derived airway epithelial cells (ALI and iALI, respectively) are physiologically relevant models for respiratory virus infection studies because they can mimic the in vivo human bronchial epithelium. Here, we investigated gene expression profiles in human airway cultures (ALI and iALI models), infected or not with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), using our own and publicly available bulk and single-cell transcriptome datasets...
July 27, 2023: International Journal of Molecular Sciences
#36
Hei-Shun Cheng, Pui-Hing Chiu, Charles Wong, Chun-Wai Tong, Pui-Ling Flora Miu
Adult-onset immunodeficiency (AOID) syndrome due to the presence of anti-interferon gamma antibody (AIGA) is characterized by multiple opportunistic infections. We report a case of a 65-year old healthy woman who suffered from Legionella pneumophila and Mycobacterium intracellulare co-infection with clinical presentation mimicking metastatic lung cancer. She presented with chronic cough and weight loss. Her positron emission tomography scan showed a right upper lobe mass, mediastinal lymphadenopathy and multiple bone lesions...
September 2023: Respirology Case Reports
#37
JOURNAL ARTICLE
Stejara A Netea, Giske Biesbroek, Diana van Stijn, Hanna Ijspeert, Caspar I van der Made, Machiel H Jansen, Judy Geissler, J M Merlijn van den Berg, Martijn van der Kuip, Mariken P Gruppen, Dieneke Schonenberg-Meinema, Berber Kapitein, A M Marceline Tutu van Furth, Sietse Q Nagelkerke, Dasja Pajkrt, Frans B Plötz, M E J Lisette den Boer, Gijs W Landman, Marlies A van Houten, Ines Goetschalckx, Erik J M Toonen, Frank L van de Veerdonk, Irene M Kuipers, Willem A Dik, Taco W Kuijpers
BACKGROUND: Children with SARS-CoV-2 related Multisystem Inflammatory Syndrome in Children (MIS-C) often present with clinical features that resemble Kawasaki disease (KD). Disease severity in adult COVID-19 is associated to the presence of anti-cytokine autoantibodies (ACAAs) against type I interferons. Similarly, ACAAs may be implicated in KD and MIS-C. Therefore, we explored the immunological response, presence of ACAAs and disease correlates in both disorders. METHODS: Eighteen inflammatory plasma protein levels and seven ACAAs were measured in KD (n = 216) and MIS-C (n = 56) longitudinally by Luminex and/or ELISA...
July 29, 2023: EBioMedicine
#38
JOURNAL ARTICLE
Zhennan Liao, Dandan Li, Shengyou Liao, Zhipeng Zeng, Jiayi Liu, Ting Xie, Bo Hu, Wei Wang, Xiaoping Hong, Dongzhou Liu, Lianghong Yin, Donge Tang, Yong Dai
Primary Sjogren's Syndrome (pSS) is a chronic autoimmune disease, with unclear pathogenies. Lysine-malonylation (Kmal) as a novel post-translational modification (PTMs) was found associated with metabolic, immune, and inflammatory processes. For purpose of investigating the proteomic profile and functions of kmal in pSS, liquid chromatography-tandem mass spectrometry (LC-MS/MS)-based analysis and bioinformatics analysis are performed based on twenty-eight pSS patients versus twenty-seven healthy controls (HCs)...
July 21, 2023: Journal of Proteomics
#39
JOURNAL ARTICLE
Cheng Zu, Shenghao Wu, Mingming Zhang, Guoqing Wei, Huijun Xu, Jiazhen Cui, Alex H Chang, He Huang, Yongxian Hu
BACKGROUND AIMS: With the increasing application of chimeric antigen receptor (CAR)-T cell therapy in various malignancies, an extra toxicity profile has been revealed, including a severe complication resembling hemophagocytic lymphohistiocytosis (HLH), which is usually disguised by severe cytokine release syndrome (CRS). METHODS: In a clinical trial in whom 99 patients received B-cell maturation antigen CAR-T cells, we identified 20 (20.20%) cases of CAR-T cell-associated HLH (carHLH), most of whom possessed a background of severe CRS (grade ≥3)...
July 20, 2023: Cytotherapy
#40
JOURNAL ARTICLE
Thaís Armangué, Gemma Olivé-Cirera, Eugenia Martínez-Hernandez, Maria Rodes, Vicente Peris-Sempere, Mar Guasp, Raquel Ruiz, Eduard Palou, Azucena González, Ma Ángeles Marcos, María Elena Erro, Luis Bataller, Íñigo Corral-Corral, Jesus Planagumà, Eva Caballero, Alexandru Vlagea, Jie Chen, Paul Bastard, Marie Materna, Astrid Marchal, Laurent Abel, Aurélie Cobat, Laia Alsina, Clàudia Fortuny, Albert Saiz, Emmanuel Mignot, Adeline Vanderver, Jean-Laurent Casanova, Shen-Ying Zhang, Josep Dalmau
Patients with herpes simplex virus (HSV) encephalitis (HSE) often develop neuronal autoantibody-associated encephalitis (AE) post-infection. Risk factors of AE are unknown. We tested the hypotheses that predisposition for AE post-HSE may be involved, including genetic variants at specific loci, human leucocyte (HLA) haplotypes, or the blood innate immune response against HSV, including type I interferon (IFN) immunity. Patients of all ages with HSE diagnosed between 1 January 2014 and 31 December 2021 were included in one of two cohorts depending on whether the recruitment was at HSE onset (Spanish Cohort A) or by the time of new neurological manifestations (international Cohort B)...
October 3, 2023: Brain
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