Article
Hematology
Akshay Sharma, Alexis Leonard, Kamille West, Jeffrey M. Gossett, Naoya Uchida, Sandhya Panch, David Stroncek, Leigh Poston, Salem Akel, Jane S. Hankins, Courtney Fitzhugh, Matthew M. Hsieh, Guolian Kang, John F. Tisdale, Mitchell J. Weiss, Yan Zheng
Summary: We adjusted the collection strategy for haematopoietic stem and progenitor cells from patients with sickle cell disease, resulting in improved collection efficiency. However, this modification also led to an increase in red blood cell contamination of the collected cells.
BRITISH JOURNAL OF HAEMATOLOGY
(2022)
Article
Multidisciplinary Sciences
Gregory A. Newby, Jonathan S. Yen, Kaitly J. Woodard, Thiyagaraj Mayuranathan, Cicera R. Lazzarotto, Yichao Li, Heather Sheppard-Tillman, Shaina N. Porter, Yu Yao, Kalin Mayberry, Kelcee A. Everette, Yoonjeong Jang, Christopher J. Podracky, Elizabeth Thaman, Christophe Lechauve, Akshay Sharma, Jordana M. Henderson, Michelle F. Richter, Kevin T. Zhao, Shannon M. Miller, Tina Wang, Luke W. Koblan, Anton P. McCaffrey, John F. Tisdale, Theodosia A. Kalfa, Shondra M. Pruett-Miller, Shengdar Q. Tsai, Mitchell J. Weiss, David R. Liu
Summary: The study demonstrated successful conversion of sickle cell disease allele into a non-pathogenic variant using adenine base editor, with durable therapeutic effects. The edited HSPCs improved physiological parameters and reduced pathological abnormalities in spleens of mice, indicating the potential for long-lasting and effective treatment for SCD.
Article
Cell Biology
Dehao Huang, Qianhao Zhao, Mengyun Zhang, Qitong Weng, Qi Zhang, Kaitao Wang, Fang Dong, Hui Cheng, Fangxiao Hu, Jinyong Wang
Summary: In this study, the researchers used a mouse model with conditional expression of Hoxb5 to investigate its effect on multipotent blood progenitor cells. They found that induced expression of Hoxb5 in these cells led to the generation of a new cell type that can repopulate long-term multilineage haematopoiesis. RNA-seq analysis showed that these cells exhibited features of DNA replication and cell division.
CELL PROLIFERATION
(2022)
Article
Hematology
Alexis Leonard, Akshay Sharma, Naoya Uchida, David Stroncek, Sandhya R. Panch, Kamille West, Eoghan Molloy, Thomas E. Hughes, Sara Hauffe, Tiffani Taylor, Courtney Fitzhugh, Jane S. Hankins, Megan Wilson, Shengdar Q. Tsai, Mitchell J. Weiss, Matthew Hsieh, John F. Tisdale
Summary: Recent studies have shown that plerixafor mobilization and apheresis in patients with sickle cell disease can safely collect sufficient CD34(+) hematopoietic stem cells for clinical gene therapy applications. The quantity of CD34(+) cells mobilized by plerixafor varies among SCD patients for unknown reasons, and factors influencing this yield include age, baseline and pre-apheresis CD34(+) cell counts, and disease severity markers unique to SCD.
Article
Cell Biology
Joey J. Ghersi, Gabriel Baldissera, Jared Hintzen, Stephanie A. Luff, Siyuan Cheng, Ivan Fan Xia, Christopher M. Sturgeon, Stefania Nicoli
Summary: Ghersi et al. have found that the diversity of haematopoietic stem and progenitor cells is established at the haemogenic endothelium level and is regulated by microRNA-128-mediated modulation of Wnt and Notch signalling. Haematopoietic stem and progenitor cells (HSPCs) generate different lineages and are produced in the embryo through the transformation of haemogenic endothelial cells in the aorta-gonad-mesonephros (AGM). The researchers discovered that loss of microRNA-128 leads to an expansion of HSPCs in the AGM with different cell cycle states and a bias towards erythroid and lymphoid progenitors.
NATURE CELL BIOLOGY
(2023)
Article
Multidisciplinary Sciences
Isabell Hess, Sagar, Connor O'Meara, Dominic Gruen, Michael Schorpp, Thomas Boehm
Summary: The zinc finger transcription factor Ikzf1 plays an important role in lymphoid development in mammals. This study investigates the phenotypes of zebrafish homozygous for two different mutant ikzf1 alleles. The results show that the mutants exhibit increased myelopoiesis and stimulate erythroid differentiation, suggesting a stage-specific function of ikzf1 in regulating hematopoiesis.
SCIENTIFIC REPORTS
(2022)
Article
Cell Biology
Ismael Morin-Poulard, Manon Destalminil-Letourneau, Laetitia Bataille, Jean-Louis Frendo, Gaelle Lebreton, Nathalie Vanzo, Michele Crozatier
Summary: This study investigates the ontogeny of the posterior lobes of the Drosophila lymph gland during larval development and identifies the genetic basis of this process. The posterior lobes are found to originate from embryonic pericardial cells, and their composition of blood progenitors is progressively built during larval development. Homeotic genes and the transcription factor Klf15 are found to regulate the fate choice between blood cell and nephrocyte in larvae.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2022)
Article
Rheumatology
Darja Andreev, Mengdan Liu, Katerina Kachler, Mireia Llerins Perez, Philipp Kirchner, Julia Koelle, Andreas Giessl, Simon Rauber, Rui Song, Oliver Aust, Anika Grueneboom, Arnd Kleyer, Juan D. Canete, Arif Ekici, Andreas Ramming, Susetta Finotto, Georg Schett, Aline Bozec
Summary: Research suggests the existence of a proresolving eosinophil subset in eosinophilic asthma, which may protect joints in arthritis. These regulatory eosinophils (rEos) in the joints expand due to systemic upregulation of IL-5 released by lung ILC2s.
ANNALS OF THE RHEUMATIC DISEASES
(2021)
Editorial Material
Hematology
Laura McLaughlin, Edwin DeZoeten, Michael R. Verneris
Summary: The article by Moser et al. discusses the outcomes of allogeneic transplantation in 11 patients with immune deficiency syndrome and inflammatory bowel disease (IBD), showing low rates of complications and resolution of symptoms. The study suggests that allogeneic transplantation may be a promising option for IBD treatment.
BRITISH JOURNAL OF HAEMATOLOGY
(2023)
Article
Physics, Multidisciplinary
Viviana Claveria, Philippe Connes, Luca Lanotte, Celine Renoux, Philippe Joly, Romain Fort, Alexandra Gauthier, Christian Wagner, Manouk Abkarian
Summary: Red blood cells in sickle cell anemia exhibit more heterogeneous physical properties compared to healthy red blood cells, with differences in adhesiveness, rigidity, density, size, and shape. The segregation of sickle red blood cells in micron-sized channels was investigated, with findings showing that low-density cells tended to remain in the center of the channel while the densest cells segregated towards the walls. Aggregation inhibited segregation and increased the cell depleted layer thickness, potentially playing a protective role against vaso-occlusion in sickle cell anemia patients.
FRONTIERS IN PHYSICS
(2021)
Article
Immunology
Luciana Ribeiro Jarduli-Maciel, Julia Teixeira Cottas de Azevedo, Emmanuel Clave, Thalita Cristina de Mello Costa, Lucas Coelho Marliere Arruda, Isabelle Fournier, Patricia Vianna Bonini Palma, Keli Cristina Lima, Juliana Bernardes Elias, Ana Beatriz P. L. Stracieri, Fabiano Pieroni, Renato Cunha, Luiz Guilherme Darrigo-Junior, Carlos Eduardo Settani Grecco, Dimas Tadeu Covas, Ana Cristina Silva-Pinto, Gil Cunha De Santis, Belinda Pinto Simoes, Maria Carolina Oliveira, Antoine Toubert, Kelen Cristina Ribeiro Malmegrim
Summary: In this study, the reconstitution of T- and B-cell compartments in SCD patients treated with allo-HSCT was comprehensively evaluated. The occurrence of acute graft-versus-host disease transiently affected the reconstitution of T- and B-cells. In addition, an increase in IL-10-producing B-regulatory cells was observed after transplantation, which may contribute to improved immune regulation and homeostasis.
CLINICAL & TRANSLATIONAL IMMUNOLOGY
(2022)
Review
Urology & Nephrology
Kenneth Ataga, Santosh L. Saraf, Vimal K. Derebail
Summary: Sickle cell syndromes are associated with kidney abnormalities and nephropathy. The pathophysiology of SCD-related nephropathy is multifactorial, including oxidative stress, hyperfiltration, and glomerular hypertension. Patients with SCD experience faster decline in kidney function and often present with albuminuria. Multiple genetic modifiers are also implicated in the development and progression of SCD-related nephropathy. Chronic kidney disease and rapid decline in estimated glomerular filtration rate are associated with increased mortality in SCD patients.
NATURE REVIEWS NEPHROLOGY
(2022)
Article
Biochemical Research Methods
Endris Muhammed, James Cooper, Daniel Devito, Robert Mushi, Maria del Pilar Aguinaga, Daniel Erenso, Horace Crogman
Summary: The study introduces a model for better calibration of trapping force by applying an oppositely directed drag force on red blood cells, and analyzes the elastic properties of sickle cell anemia and sickle cell trait blood samples. Results indicate that sickle cell trait samples exhibit higher elastic properties compared to sickle cell anemia samples, possibly due to differences in membrane lipid composition affected by cholesterol concentrations.
JOURNAL OF BIOMEDICAL OPTICS
(2021)
Article
Clinical Neurology
Kemar Prussien, Bruce E. Compas, Rachel E. Siciliano, Abagail E. Ciriegio, Chelsea A. Lee, Adetola A. Kassim, Michael R. DeBaun, Manus J. Donahue, Lori C. Jordan
Summary: Elevated cerebral blood flow and oxygen extraction fraction in individuals with sickle cell anemia are associated with deficits in executive function, with significant correlations remaining in multivariate analyses. This may be attributed to hemodynamic impairment.
Article
Multidisciplinary Sciences
Maxime Darrin, Ashwin Samudre, Maxime Sahun, Scott Atwell, Catherine Badens, Anne Charrier, Emmanuele Helfer, Annie Viallat, Vincent Cohen-Addad, Sophie Giffard-Roisin
Summary: The fraction of red blood cells adopting a specific motion under low shear flow can be used as a promising marker for monitoring the clinical status of patients with sickle cell disease. A two-stage machine learning pipeline is presented to automatically classify cell motions in videos, eliminating unreliable samples and discriminating between highly and poorly deformable cells. The pipeline achieves a high accuracy of 97% and an F1-score of 0.94. Dataset and codes are publicly available.
SCIENTIFIC REPORTS
(2023)
