Article
Chemistry, Multidisciplinary
Deogil Kim, Byung-Hyun Cha, Jinsung Ahn, Yoshie Arai, Bogyu Choi, Soo-Hong Lee
Summary: Understanding the biophysical relationships between stem cells and applied biomaterials is crucial in controlling stem cell functions. This study highlights the significant role of 3D microenvironment, particularly methacrylated hyaluronic acid (HA) hydrogel, in improving cellular reprogramming into iPSCs. The incorporation of cues from the 3D microenvironment accelerates the reprogramming process, offering potential advantages for translational applications.
ADVANCED FUNCTIONAL MATERIALS
(2021)
Article
Multidisciplinary Sciences
Jingyang Guan, Guan Wang, Jinlin Wang, Zhengyuan Zhang, Yao Fu, Lin Cheng, Gaofan Meng, Yulin Lyu, Jialiang Zhu, Yanqin Li, Yanglu Wang, Shijia Liuyang, Bei Liu, Zirun Yang, Huanjing He, Xinxing Zhong, Qijing Chen, Xu Zhang, Shicheng Sun, Weifeng Lai, Yan Shi, Lulu Liu, Lipeng Wang, Cheng Li, Shichun Lu, Hongkui Deng
Summary: This study demonstrates the chemical reprogramming of human somatic cells to human chemically induced pluripotent stem cells, which exhibit key features of embryonic stem cells. The induction of an intermediate plastic state and inhibition of the JNK pathway are crucial for successful chemical reprogramming.
Review
Endocrinology & Metabolism
Ila Tewari Jasra, Nerea Cuesta-Gomez, Kevin Verhoeff, Braulio A. Marfil-Garza, Nidheesh Dadheech, A. M. James Shapiro
Summary: This review summarizes the roles and mechanisms of mitochondria in somatic cell reprogramming to iPSCs and the metabolic shift associated with directed differentiation into pancreatic beta-like cells.
FRONTIERS IN ENDOCRINOLOGY
(2023)
Article
Cell & Tissue Engineering
Sandhya Sriram, Nam-Young Kang, Subha Subramanian, Tannistha Nandi, Samydurai Sudhagar, Qiaorui Xing, Gerine Jin-Ling Tong, Allen Kuan-Liang Chen, Thekkeparambil Chandrabose Srijaya, Patrick Tan, Yuin-Han Loh, Young-Tae Chang, Shigeki Sugii
Summary: In this study, a novel BODIPY-derived fluorescent probe, BDL-E5, was identified to detect live human iPS cells at the early reprogramming stage, enabling early identification of reprogramming cells and improving the efficiency of iPS cell generation. RNA sequencing analysis revealed early reprogramming gene expression patterns, particularly involving CREB1, which was found to significantly impact reprogramming efficiency when overexpressed or knocked down. Overall, BDL-E5 provides a valuable tool for studying the early reprogramming pathway and enhancing the production of human iPS cells for clinical applications.
STEM CELL RESEARCH & THERAPY
(2021)
Article
Cell & Tissue Engineering
Ana Belen Alvarez-Palomo, Jordi Requena-Osete, Raul Delgado-Morales, Victoria Moreno-Manzano, Carme Grau-Bove, Agueda M. Tejera, Manel Juan Otero, Carme Barrot, Irene Santos-Barriopedro, Alejandro Vaquero, Jovita Mezquita-Pla, Sebastian Moran, Carlos Hobeich Naya, Iris Garcia-Martinez, Francisco Vidal Perez, Maria A. Blasco, Manel Esteller, Michael J. Edel
Summary: The study demonstrates that utilizing synthetic mRNA transfection of CYCLIN D1 during reprogramming repairs DNA and significantly improves the genetic stability of human iPSC. This method reduces various genetic instabilities, decreases DNA damage, promotes correct protein expression, and reduces single-nucleotide polymorphism changes per chromosome.
Article
Cell Biology
Ying Zhang, Jun Wei, Jiani Cao, Kehua Zhang, Yaojin Peng, Hongkui Deng, Jiuhong Kang, Guangjin Pan, Yong Zhang, Boqiang Fu, Shijun Hu, Jie Na, Yan Liu, Lei Wang, Lingmin Liang, Huanxin Zhu, Yu Zhang, Zi-Bing Jin, Jie Hao, Aijin Ma, Tongbiao Zhao, Junying Yu
Summary: "Requirements for Human-Induced Pluripotent Stem Cells" is the first set of guidelines in China on human-induced pluripotent stem cells. It provides detailed technical requirements and aims to promote international standardization.
CELL PROLIFERATION
(2022)
Article
Engineering, Biomedical
Hong Cao, Qian Zhou, Chungeng Liu, Yecen Zhang, Minghui Xie, Weihua Qiao, Nianguo Dong
Summary: This study investigates the effect of substrate stiffness on the differentiation of human-induced pluripotent stem cells (h-iPSCs) into valve endothelial-like cells (h-iVECs). The results show that moderate substrate stiffness can enhance the differentiation efficiency of h-iVECs and is regulated by the WNT/Calcineurin signaling pathway. This study emphasizes the importance of using substrate stiffness to achieve a more specific and mature differentiation of h-iVECs.
ACTA BIOMATERIALIA
(2022)
Article
Biochemical Research Methods
Julia Vallverdu, Raquel A. Martinez Garcia de la Torre, Inge Mannaerts, Stefaan Verhulst, Ayla Smout, Mar Coll, Silvia Arino, Teresa Rubio-Tomas, Beatriz Aguilar-Bravo, Celia Martinez-Sanchez, Delia Blaya, Catherine M. Verfaillie, Leo A. van Grunsven, Pau Sancho-Bru
Summary: Human iPSCs are differentiated into HSCs with growth factors for in vitro modeling. The protocol yields iPSC-HSCs with phenotypic and functional characteristics of primary HSCs, suitable for high-throughput in vitro studies. Coculturing iPSC-HSCs with hepatocytes allows for the formation of 3D hepatic spheroids, enabling modeling and drug screening studies.
Article
Medicine, Research & Experimental
Dhanya Menon, Smitha Bhaskar, Preethi Sheshadri, Chaitanya G. Joshi, Darshan Patel, Anujith Kumar
Summary: The study found that canine iPSCs exhibit characteristics of both naive and primed pluripotent stem cells, representing a unique intermediary state of pluripotency distinct from that of mice and human pluripotent stem cells.
Article
Biochemistry & Molecular Biology
Yue Su, Ling Wang, Zhiqiang Fan, Ying Liu, Jiaqi Zhu, Deborah Kaback, Julia Oudiz, Tayler Patrick, Siu Pok Yee, Xiuchun (Cindy) Tian, Irina Polejaeva, Young Tang
Summary: This study successfully generated biPSCs with long-term self-renewal capacity and pluripotent characteristics from bovine mesenchymal stem cells. The cells exhibited morphological changes under different culture conditions, and transcriptome analysis showed high similarity to bovine ESCs and embryos. The naive-like biPSCs also demonstrated the ability to integrate into extra-embryonic tissues.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Immunology
Niclas Flosdorf, Martin Zenke
Summary: Novel methods of generating dendritic cells (DC) through reprogramming somatic cells have opened up exciting avenues for research and clinical applications. Induced pluripotent stem cells (iPS cells) and direct reprogramming of cells can both be used to generate DC.
EUROPEAN JOURNAL OF IMMUNOLOGY
(2022)
Review
Biochemistry & Molecular Biology
Tianyuan Shi, Martin Cheung
Summary: Using rodents for modeling neurological diseases has limitations due to differences in genetics, neural development, and physiology between humans and rodents. In the past decade, induced pluripotent stem cells and induced neural stem cells generated through reprogramming somatic cells have offered a powerful alternative for studying disease pathogenesis and testing regenerative medicines. Urine-derived stem cells are an ideal cell source for reprogramming due to their proliferation, multipotency, epithelial nature, and ease of reprogramming.
CELL AND BIOSCIENCE
(2021)
Review
Cell Biology
Samantha E. Spellicy, David C. Hess
Summary: Inflammation is a key contributing factor to the pathogenesis of stroke, leading to increased oxidative stress and neuronal cell death. Recent research has shown that induced pluripotent stem cells have the potential to suppress neuroinflammation, mainly by modulating inflammatory signaling and endogenous inflammatory cell populations.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Cell Biology
Taiki Satoh, Marcelo A. S. Toledo, Janik Boehnke, Kathrin Olschok, Niclas Flosdorf, Katrin Goetz, Caroline Kuestermann, Stephanie Sontag, Kristin Sere, Steffen Koschmieder, Tim H. Bruemmendorf, Nicolas Chatain, Yoh-ichi Tagawa, Martin Zenke
Summary: Dendritic cells (DC) are professional antigen-presenting cells that can be divided into different subsets, with the DC3 subset having proinflammatory properties. Human iPS cell-derived DC3 exhibit similar characteristics to blood DC3 in vitro. The JAK2 V617F mutation enhances DC3 production and biases towards erythrocytes and megakaryocytes.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Neurosciences
Dwaipayan Adhya, Vivek Swarup, Roland Nagy, Lucia Dutan, Carole Shum, Eva P. Valencia-Alarcon, Kamila Maria Jozwik, Maria Andreina Mendez, Jamie Horder, Eva Loth, Paulina Nowosiad, Irene Lee, David Skuse, Frances A. Flinter, Declan Murphy, Grainne McAlonan, Daniel H. Geschwind, Jack Price, Jason Carroll, Deepak P. Srivastava, Simon Baron-Cohen
Summary: The study suggests that autism-iPSCs diverge from control-iPSCs at a cellular level during early neurodevelopment, indicating unique developmental differences associated with autism may be established at early prenatal stages.
BIOLOGICAL PSYCHIATRY
(2021)
Article
Allergy
Mitra Rezaei, Majid Marjani, Alireza Mirahmadi, Niloufar Bineshfar, Hadiseh Mohammadpour, Mihan Pourabdollah Toutkaboni, Farinaz Nasr Azadani, Zahra Amanzade, Payam Tabarsi, Seyed Ali Ziai, Jean Laurent Casanova, Davood Mansouri
Summary: This study found that there was a significant negative correlation between age and STING mRNA expression level in COVID-19 patients. Patients with severe illness, admitted to the ICU, or who eventually died had lower STING expression levels.
INTERNATIONAL ARCHIVES OF ALLERGY AND IMMUNOLOGY
(2023)
Article
Immunology
Ana Karen Penafiel Vicuna, Marco Yamazaki Nakashimada, Ximena Leon Lara, Elizabeth Mendieta Flores, Maria Enriqueta Nunez Nunez, Juan Carlos Lona-Reyes, Leticia Hernandez Nieto, Maria Guadalupe Ramirez Vazquez, Joel Barroso Santos, Alvaro Lopez Iniguez, Yolanda Gonzalez, Martha Torres, Jose Luis Lezana Fernandez, Carla M. Roman Montes, Edgar Alejandro Medina-Torres, Edith Gonzalez Serrano, Juan Carlos Bustamante Ogando, Saul Lugo Reyes, Oscar Zavaleta Martinez, Aide Tamara Staines Boone, Edna Venegas Montoya, Nancy Evelyn Aguilar Gomez, Camille Soudee, Emmanuelle Jouanguy, Anne Puel, Stephanie Boisson-Dupuis, Sigifredo Pedraza Sanchez, Jean-Laurent Casanova, Francisco Espinosa Rosales, Sara Espinosa Padilla, Jacinta Bustamante, Lizbeth Blancas Galicia
Summary: This study reviewed the clinical, immunological, and genetic characteristics of MSMD patients in Mexico and identified IL12RB1 gene defect as the main cause of MSMD in the cohort. BCG infection was found to be the primary cause of morbidity and mortality in these patients.
JOURNAL OF CLINICAL IMMUNOLOGY
(2023)
Article
Immunology
Ilad Alavi Darazam, Atousa Hakamifard, Mana Momenilandi, Marie Materna, Farid Javandoust Gharehbagh, Mohammad Shahrooei, Nasrin Alipour Olyaei, Farahnaz Bidari Zerehpoosh, Antoine Fayand, Firouze Hatami, Legha Lotfollahi, Nahal Mansouri, Jean-Laurent Casanova, Vivien Beziat, Davood Mansouri
Summary: TAP2 deficiency can lead to MHC-I deficiency, causing immunodeficiency disorder. Two Iranian cases with chronic necrotizing granulomatous skin lesions due to TAP2 deficiency were reported, and three additional asymptomatic carriers were identified in their family members. Immunophenotyping revealed abnormal immune cell counts in the patients.
JOURNAL OF CLINICAL IMMUNOLOGY
(2023)
Article
Immunology
Cecilia B. Korol, Serkan Belkaya, Fahad Alsohime, Lazaro Lorenzo, Stephanie Boisson-Dupuis, Joseph Brancale, Anna-Lena Neehus, Silvia Vilarinho, Alsum Zobaida, Rabih Halwani, Saleh Al-Muhsen, Jean-Laurent Casanova, Emmanuelle Jouanguy
Summary: Fulminant viral hepatitis (FVH) caused by hepatitis A virus (HAV) is a life-threatening disease. Inherited IL-10RB deficiency and possibly inherited IL-10 and IL-10RA deficiencies confer a predisposition to FVH. Vaccination is recommended for patients with these deficiencies.
JOURNAL OF CLINICAL IMMUNOLOGY
(2023)
Article
Immunology
Ilona Gottschalk, Uwe Koelsch, Dimitrios L. Wagner, Jonas Kath, Stefania Martini, Renate Krueger, Anne Puel, Jean-Laurent Casanova, Aleksandra Jezela-Stanek, Rainer Rossi, Salima El Chehadeh, Hilde Van Esch, Horst von Bernuth
Summary: Patients with MECP2/IRAK1 duplication syndrome do not show excessive NF-kappa B signaling, suggesting that therapeutic suppression of this pathway may not benefit these patients.
JOURNAL OF CLINICAL IMMUNOLOGY
(2023)
Article
Medicine, Research & Experimental
Nasrinsadat Nabavizadeh, Annkatrin Bressin, Mohammad Shboul, Ricardo Moreno Traspas, Poh Hui Chia, Carine Bonnard, Emmanuelle Szenker-Ravi, Burak Saribas, Emmanuel Beillard, Umut Altunoglu, Zohreh Hojati, Scott Drutman, Susanne Freier, Mohammad El-Khateeb, Rajaa Fathallah, Jean-Laurent Casanova, Wesam Soror, Alaa Arafat, Nathalie Escande-Beillard, Andreas Mayer, Bruno Reversade
Summary: Exome sequencing has revolutionized the identification of germline variations responsible for Mendelian diseases, but it cannot capture non-coding regions. This study demonstrates how RNA-seq can complement exome sequencing by identifying a deep intronic mutation in a gene associated with Osteogenesis Imperfecta and neonatal progeria syndrome. The mutation alters pre-mRNA processing and leads to exon skipping, revealing the importance of transcriptomic approaches in understanding the molecular mechanisms of human diseases.
EMBO MOLECULAR MEDICINE
(2023)
Article
Clinical Neurology
Alessia Giossi, Silvia Clara Giliani, Massimo Gamba, Paola Toniati, Mauro Magoni, Alessandro Pezzini
Summary: This article describes a case of an adult patient with recurrent acute ischemic strokes due to cerebral small vessel disease (cSVD), without any other phenotypic manifestations. Targeted next-generation sequencing detected a pathogenic missense variant in the ADA2 gene (c.139G > A, p.G47R), consistent with the diagnosis of adenosine deaminase 2 deficiency syndrome. The findings highlight the diagnostic potential of next-generation sequencing for identifying genetic causes in stroke patients with atypical presentations of cSVD.
EUROPEAN JOURNAL OF NEUROLOGY
(2023)
Article
Immunology
Abderrahmane Errami, Jamila El Baghdadi, Fatima Ailal, Ibtihal Benhsaien, Jalila El Bakkouri, Leila Jeddane, Noureddine Rada, Noufissa Benajiba, Khaoula Mokhantar, Kaoutar Ouazahrou, Sanae Zaidi, Laurent Abel, Jean-Laurent Casanova, Stephanie Boisson-Dupuis, Jacinta Bustamante, Ahmed Aziz Bousfiha
Summary: We studied the genetic predisposition to mycobacterial diseases in Moroccan kindreds through clinical, immunological, and genetic analysis. The study revealed a Mendelian susceptibility to mycobacterial disease (MSMD) in these families, leading to severe tuberculosis and complications of BCG vaccination. Systematic genetic studies are necessary in the presence of these infections, and BCG vaccination should be delayed in newborn siblings until a genetic predisposition to mycobacteria is excluded.
JOURNAL OF CLINICAL IMMUNOLOGY
(2023)
Review
Allergy
Estefania Vasquez-Echeverri, Marco Antonio Yamazaki-Nakashimada, Edna Venegas Montoya, Selma Cecilia Scheffler Mendoza, Lina Maria Castano-Jaramillo, Edgar Alejandro Medina-Torres, Maria Edith Gonzalez-Serrano, Melissa Espinosa-Navarro, Juan Carlos Bustamante Ogando, Maria Guadalupe Gonzalez-Villarreal, Margarita Ortega Cisneros, Pedro Francisco Valencia Mayoral, Alejandra Consuelo Sanchez, Gustavo Varela-Fascinetto, Rosa Maria Nideshda Ramirez-Uribe, Yuridia Salazar Galvez, Laura Cecilia Bonifaz Alonzo, Ezequiel Moises Fuentes-Panana, Noemi Gomez Hernandez, Cesar Mauricio Rojas Maruri, Jean-Laurent Casanova, Sara Elva Espinosa-Padilla, Aide Tamara Staines Boone, Gabriel Lopez-Velazquez, Bertrand Boisson, Saul Oswaldo Lugo Reyes
Summary: This study describes the clinical, laboratory, and genetic features of six patients from four Mexican families with hereditary actin-related protein 2/3 complex subunit 1B deficiency. The results expand the clinical spectrum of the disease and suggest possible genetic causes.
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE
(2023)
Article
Cell Biology
Steicy Sobrino, Alessandra Magnani, Michaela Semeraro, Loredana Martignetti, Akira Cortal, Adeline Denis, Chloe Couzin, Capucine Picard, Jacinta Bustamante, Elisa Magrin, Laure Joseph, Cecile Roudaut, Aurelie Gabrion, Tayebeh Soheili, Corinne Cordier, Olivier Lortholary, Francois Lefrere, Frederic Rieux-Laucat, Jean-Laurent Casanova, Sylvain Bodard, Nathalie Boddaert, Adrian J. Thrasher, Fabien Touzot, Sophie Taque, Felipe Suarez, Ambroise Marcais, Agathe Guilloux, Chantal Lagresle-Peyrou, Anne Galy, Antonio Rausell, Stephane Blanche, Marina Cavazzana, Emmanuelle Six
Summary: X-linked chronic granulomatous disease (CGD) is a serious condition associated with defective phagocytosis, infections, and inflammatory complications. A clinical trial of lentivirus-based gene therapy was conducted on four patients, showing successful engraftment and clinical benefits in two patients, while the other two experienced loss of gene-corrected cells. Single-cell transcriptomic analysis revealed lower levels of hematopoietic stem cells (HSCs) in CGD patients, especially in those with poor engraftment. Aberrant HSC state and elevated interferon genes were identified as predictors of HSC engraftment failure.
CELL REPORTS MEDICINE
(2023)
Article
Cardiac & Cardiovascular Systems
Can Gollmann-Tepekoeylue, Michael Graber, Jakob Hirsch, Sophia Mair, Andreas Naschberger, Leo Poelzl, Felix Naegele, Elke Kirchmair, Gerald Degenhart, Egon Demetz, Richard Hilbe, Hao-Yu Chen, James C. Engert, Anna Boehm, Nadja Franz, Daniela Lobenwein, Daniela Lener, Christiane Fuchs, Anna Weihs, Sonja Toechterle, Georg F. Vogel, Victor Schweiger, Jonas Eder, Peter Pietschmann, Markus Seifert, Florian Kronenberg, Stefan Coassin, Michael Blumer, Hubert Hackl, Dirk Meyer, Gudrun Feuchtner, Rudolf Kirchmair, Jakob Troppmair, Markus Krane, Guenther Weiss, Sotirios Tsimikas, George Thanassoulis, Michael Grimm, Bernhard Rupp, Lukas A. Huber, Shen-Ying Zhang, Jean-Laurent Casanova, Ivan Tancevski, Johannes Holfeld
Summary: TLR3 is identified as a central molecular regulator of calcification in valvular interstitial cells, and BGN is revealed as a new endogenous agonist of TLR3. BGN activates TLR3 through XYLT1-dependent posttranslational modification and induces the transdifferentiation of valvular interstitial cells into bone-forming osteoblasts through TLR3-dependent induction of type I interferons.
Article
Multidisciplinary Sciences
Jean-Laurent Casanova
Summary: In 1955, Rene Dubos questioned the germ theory and proposed that various changing circumstances weaken the host and lead to infectious diseases, without specifying the mechanisms involved. He highlighted that only a small proportion of individuals infected by any microbe develop clinical disease. However, he overlooked the extensive findings reported from 1905 onward, which unequivocally demonstrated the role of host genetic determinants in infection outcomes. Over the next 50 years, additional evidence supported the genetic and immunological observations that Dubos had ignored. Immunosuppression- and HIV-driven immunodeficiencies further provided a mechanistic basis for Dubos' viewpoint. These lines of evidence collectively support a host theory of infectious diseases, where inherited and acquired immunodeficiencies are the primary factors determining severe infection outcomes, with the germ serving as an environmental trigger that reveals an underlying and preexisting cause of disease and death.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2023)
Article
Cell Biology
Justin Jacobse, Rachel E. Brown, Jing Li, Jennifer M. Pilat, Ly Pham, Sarah P. Short, Christopher T. Peek, Andrea Rolong, M. Kay Washington, Ruben Martinez-Barricarte, Mariana X. Byndloss, Catherine Shelton, Janet G. Markle, Yvonne L. Latour, Margaret M. Allaman, James E. Cassat, Keith T. Wilson, Yash A. Choksi, Christopher S. Williams, Ken S. Lau, Charles R. Flynn, Jean-Laurent Casanova, Edmond H. H. M. Rings, Janneke N. Samsom, Jeremy A. Goettel
Summary: The cytokine IL-23 plays a role in the development of inflammatory bowel disease (IBD). IL-23R is highly expressed in intestinal Tregs, but its effect on Tregs remains unknown. This study shows that IL-23 reduces the frequency and suppressive function of colonic Tregs and that mice lacking IL-23R in Tregs have increased Treg frequency and advantages during inflammation. Furthermore, IL-23 antagonizes the liver X receptor pathway, cellular cholesterol transporter Abca1, and increases Treg apoptosis. These findings suggest that IL-23 negatively regulates intestinal Tregs, potentially promoting chronic inflammation in patients with IBD.
Article
Microbiology
Assiya El Kettani, Fatima Ailal, Farida Marnissi, Fouzia Hali, Jalila El Bakkouri, Ibtihal Benhsaien, Tom Le Voyer, Mame Sokhna Gueye, Remi Chevalier, Soumiya Chiheb, Khalid Zerouali, Emmanuelle Jouanguy, Jean-Laurent Casanova, Ahmed Aziz Bousfiha
Summary: Unusual viral skin infections can be the initial clinical manifestation in children with inborn errors of immunity (IEI). Through clinical, immunological, and genetic investigations, several genetic deficiencies related to IEI were identified, which can help improve the diagnosis, prevention, and treatment for patients and their families.
Article
Engineering, Manufacturing
Rosaria Rovetta, Paola Ginestra, Rosalba Monica Ferraro, Keren Zohar-Hauber, Silvia Giliani, Elisabetta Ceretti
Summary: Laser powder bed fusion, especially selective laser melting, is used to produce near-net-shaped engineering components for biomedical applications, particularly in orthopaedics. Ti6Al4V is commonly used for orthopaedic implants due to its excellent mechanical qualities, high biocompatibility, and corrosion resistance. Surface modification is used to improve functionality and performance, with post-processing techniques such as anodization and passivation having an impact on osteoblast attachment and mineralization.
JOURNAL OF MANUFACTURING AND MATERIALS PROCESSING
(2023)
