Article
Chemistry, Medicinal
Kaluvu Balaraman, Emre Deniz, Eryn Nelson, Samantha L. Pilicer, Sezen Atasoy, Anna Molotkova, Handan Sevim, Purushottam B. Tiwari, Aykut Uren, Christian Wolf
Summary: Ewing Sarcoma (ES) is a cancer that affects the bones and soft tissues, mainly in children and young adults. CD99, a trans-membrane protein abundantly expressed on ES cells, is a diagnostic marker and a potential therapeutic target. Researchers have developed new derivatives of clofarabine and cladribine that specifically target CD99 and inhibit ES cell growth. These findings provide a roadmap for the future development of selective CD99 inhibitors for targeted treatment of ES.
EUROPEAN JOURNAL OF MEDICINAL CHEMISTRY
(2023)
Article
Chemistry, Multidisciplinary
Ling Tao, Myrthala Moreno-Smith, Rodrigo Ibarra-Garcia-Padilla, Giorgio Milazzo, Nathan A. Drolet, Blanca E. Hernandez, Young S. Oh, Ivanshi Patel, Jean J. Kim, Barry Zorman, Tajhal Patel, Abu Hena Mostafa Kamal, Yanling Zhao, John Hicks, Sanjeev A. Vasudevan, Nagireddy Putluri, Cristian Coarfa, Pavel Sumazin, Giovanni Perini, Ronald J. Parchem, Rosa A. Uribe, Eveline Barbieri
Summary: This study shows that CHAF1A gain-of-function supports cell malignancy, blocks neuronal differentiation, and promotes NB oncogenesis. Mechanistically, CHAF1A upregulates polyamine metabolism, which blocks neuronal differentiation and promotes cell cycle progression.
Article
Oncology
Alessandra De Feo, Laura Pazzaglia, Lisa Ciuffarin, Fabio Mangiagli, Michela Pasello, Elisa Simonetti, Evelin Pellegrini, Cristina Ferrari, Giuseppe Bianchi, Benedetta Spazzoli, Katia Scotlandi
Summary: Ewing's sarcoma, a common pediatric bone tumor, relies on genetic and epigenetic alterations that induce gene expression reprogramming. The study identifies miR-214-3p as a common mediator of EWS-FLI1 and CD99, and its restoration inhibits tumor cell growth and migration by repressing HMGA1 expression.
Article
Multidisciplinary Sciences
Murshed H. Sarkar, Ryoji Yagi, Yukihiro Endo, Ryo Koyama-Nasu, Yangsong Wang, Ichita Hasegawa, Toshihiro Ito, Ilkka S. Junttila, Jinfang Zhu, Motoko Y. Kimura, Toshinori Nakayama
Summary: IFN gamma suppresses Th2 cell proliferation by decreasing the expression of GFI1, a key molecule in this process. Deletion of Gfi1 gene in Th2 cells leads to impaired proliferation, while enforced expression of GFI1 restores defective proliferation even in the presence of IFN gamma.
Article
Biochemistry & Molecular Biology
Yi Li Wong, Toru Okubo, Eiko Uno, Kazuma Suda, Tsuyoshi Ishii
Summary: CD99 is a glycoprotein primarily expressed in immune cells. Its function in the skin remains unclear. This study investigated the role of CD99 in the skin using normal human epidermal keratinocytes (NHEK). The results suggest that CD99 is involved in maintaining skin homeostasis and initiating early differentiation.
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS
(2022)
Article
Biochemistry & Molecular Biology
Liyang Xu, Min Zhang, Lihua Shi, Xiaoli Yang, Lu Chen, Ning Cao, Anhua Lei, Ying Cao
Summary: The property of neural stemness contributes to cell tumorigenicity, and tumor phenotypic heterogeneity might be an effect of differentiation potential of neural stemness. The importance of neural genes in evolution is highlighted, indicating that tumorigenesis is a process of cells restoring a neural ground state along an evolutionary route pre-determined by the advantage of a neural state.
CELL AND BIOSCIENCE
(2021)
Article
Biotechnology & Applied Microbiology
Guoqiang Wang, Hong Wu, Peng Liang, Xiaojiao He, Dong Liu
Summary: The study showed that FUS could enhance the pro-fibrotic effect of AngII in cardiac fibroblasts by activating the TGF-beta 1/Smad signaling pathway, affecting cell proliferation, migration, and collagen synthesis. Additionally, FUS functions as an RNA-binding protein to positively regulate the expression of Pax3, which is involved in cancelling the effects of Fus knockdown on AngII-induced cells. This suggests that targeting the Fus/Pax3 axis may provide a potential therapy for relieving atrial fibrillation.
Article
Oncology
Osama Zakzok, Mohamed Elshanshory, Wael Zekri, Nahla Elsharkawy, Iman Zaky, Asmaa Salama, Ahmed Kamel, Ismail Elantably, Shebl Said
Summary: Detection of CD99(+), CD45(-) cells in peripheral blood by flow cytometry is a strong predictor for relapse, progression, and mortality in patients with Ewing sarcoma. Post-cycle 5 detection has higher sensitivity and specificity compared to baseline, making it a more reliable prognostic factor in newly diagnosed patients.
PEDIATRIC BLOOD & CANCER
(2022)
Article
Multidisciplinary Sciences
Zachary T. Olmsted, Janet L. Paluh
Summary: Researchers have successfully studied the co-development of the central and peripheral nervous systems with trunk mesendoderm in elongating multi-lineage organized (EMLO) gastruloids. They identified NCCs that differentiate to form peripheral neurons integrated with an upstream spinal cord region after day 8.
NATURE COMMUNICATIONS
(2021)
Article
Medicine, General & Internal
Qiang Shu, Jia-Nong Luo, Xiao-Ling Liu, Min Jing, Ting-Gang Mou, Fei Xie
Summary: We present a rare case of extraskeletal Ewing sarcoma involving the gastric area in a 19-year-old female patient. Early diagnosis and timely intervention are crucial for a good prognosis. Surgical resection remains the optimal treatment option for this aggressive tumor.
WORLD JOURNAL OF CLINICAL CASES
(2023)
Article
Medicine, Research & Experimental
Ashish H. Shah, Sarah R. Rivas, Tara T. Doucet-O'Hare, Vaidya Govindarajan, Catherine DeMarino, Tongguang Wang, Leonel Ampie, Yong Zhang, Yeshavanth Kumar Banasavadi-Siddegowda, Stuart Walbridge, Dragan Maric, Marta Garcia-Montojo, Robert K. Suter, Myoung-Hwa Lee, Kareem A. Zaghloul, Joseph Steiner, Abdel G. Elkahloun, Jay Chandar, Deepa Seetharam, Jelisah Desgraves, Wenxue Li, Kory Johnson, Michael E. Ivan, Ricardo J. Komotar, Mark R. Gilbert, John D. Heiss, Avindra Nath
Summary: Human endogenous retroviruses (HERVs) make up 8% of the human genome and can be reactivated in certain cancers, such as malignant gliomas. Pathological expression of the provirus HERV-K (HML-2) was found in gliomas and was associated with a cancer stem cell phenotype and poor outcomes. HML-2 played a critical role in maintaining glioblastoma stemness and tumorigenesis, as well as regulating embryonic stem cell programs. Inhibiting HML-2 expression with antiretroviral drugs reduced tumor viability and pluripotency, indicating HML-2 as a potential therapeutic target.
JOURNAL OF CLINICAL INVESTIGATION
(2023)
Article
Oncology
Bo Hong, Yi Li, Ran Yang, ShuYang Dai, Yong Zhan, Wen-Bo Zhang, Rui Dong
Summary: This study reveals that Ewing sarcoma (EwS) originates from neural crest cell lineage and the developmental trajectories of tumor cells vary. The interferon signaling pathway plays a crucial role in the tumorigenesis and tumor progression of EwS. JAK1 gene is a potential therapeutic target for EwS.
JOURNAL OF CANCER RESEARCH AND CLINICAL ONCOLOGY
(2022)
Article
Medicine, General & Internal
Ronald Peek, Lotte L. Eijkenboom, Didi D. M. Braat, Catharina C. M. Beerendonk
Summary: Short-term exposure to Everolimus has been shown to effectively eliminate Ewing's sarcoma tumor foci in ovarian tissue without causing harm to follicle morphology, survival, or early folliculogenesis. This suggests that pharmacological ex vivo purging of ovarian cortex tissue from contaminating ES tumor foci before autotransplantation may reduce the risk of reintroducing malignancy.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Endocrinology & Metabolism
Camilla Cristalli, Maria Cristina Manara, Sergio Valente, Evelin Pellegrini, Alberto Bavelloni, Alessandra De Feo, William Blalock, Elisabetta Di Bello, David Pineyro, Angelika Merkel, Manel Esteller, Oscar M. Tirado, Antonello Mai, Katia Scotlandi
Summary: This study found that the novel nonnucleoside DNMT inhibitor MC3343 can specifically deplete DNMT1 in Ewing sarcoma through a mechanism independent of DNA methylation, leading to perturbations in the cell cycle and DNA damage. The depletion of DNMT1 activates p53-dependent signaling and apoptosis in p53wt cells, while in p53 mutated cells, it leads to persistent micronuclei and increased DNA instability. Treatment with MC3343 also enhances the efficacy of other DNA damaging agents, suggesting its potential as an adjuvant therapy for Ewing sarcoma.
FRONTIERS IN ENDOCRINOLOGY
(2022)
Article
Multidisciplinary Sciences
Ana Catarina Menezes, Christabel Dixon, Anna Scholz, Rachael Nicholson, Adam Leckenby, Aleksandra Azevedo, Sarah Baker, Amanda F. Gilkes, Sara Davies, Richard L. Darley, Alex Tonks
Summary: This study demonstrates the regulatory role of RUNX3 in normal human erythropoiesis. RUNX3 has a modest impact on early erythroid growth and development, but promotes growth and inhibits terminal differentiation in late-stage erythroid development. Overexpression of RUNX3 increases colony formation in liquid culture.
SCIENTIFIC REPORTS
(2022)
Letter
Pathology
Benjamin Champion, Siaw Ming Chai, Mayank Bhandari, Dilini Gunawardena
Article
Oncology
Emanuela Palmerini, Marco Gambarotti, Antoine Italiano, Michael J. Nathenson, Ravin Ratan, Palma Dileo, Salvatore Provenzano, Robin L. Jones, Steven G. DuBois, Javier Martin-Broto, Enrique de Alava, Giacomo G. Baldi, Giovanni Grignani, Virginia Ferraresi, Antonella Brunello, Luca Paoluzzi, Rossella Bertulli, Nadia Hindi, Michael Montemurro, Christian Rothermundt, Stefania Cocchi, Carmen Salguero-Aranda, Davide Donati, Juan D. Martin, Amr H. Abdelhamid Ahmed, Alessandro Mazzocca, Elisa Carretta, Marilena Cesari, Michela Pierini, Alberto Righi, Marta Sbaraglia, Maria A. Laginestra, Katia Scotlandi, Angelo P. Dei Tos, Toni Ibrahim, Silvia Stacchiotti, Bruno Vincenzi
Summary: This study aimed to define the clinical characteristics, treatment, and outcome of undifferentiated small round cell sarcomas (URCSs) patients. The results showed significant differences in prognosis between URCS subtypes, and a full molecular assessment is needed to confirm the diagnosis. Prospective studies are needed to determine the optimal treatment strategy for each URCS subtype.
EUROPEAN JOURNAL OF CANCER
(2023)
Article
Automation & Control Systems
Ali Ahmadi Dastjerdi, Alessandro Astolfi, Niranjan Saikumar, Nima Karbasizadeh, Duarte Valerio, S. Hassan HosseinNia
Summary: This article presents a closed-loop frequency analysis tool for reset control systems. It provides sufficient conditions for the existence of steady-state response and shows that the steady-state response for periodic inputs is periodic with the same period as the input. The framework presented in this article allows for the computation of steady-state response and defines a notion of closed-loop frequency response, including high order harmonics.
IEEE TRANSACTIONS ON AUTOMATIC CONTROL
(2023)
Review
Biochemistry & Molecular Biology
Claudia Maria Hattinger, Iris Chiara Salaroglio, Leonardo Fantoni, Martina Godel, Chiara Casotti, Joanna Kopecka, Katia Scotlandi, Toni Ibrahim, Chiara Riganti, Massimo Serra
Summary: In order to improve the prognosis and cure rate of high-grade osteosarcomas (HGOSs), immune-based treatment approaches, especially for metastatic, relapsed and refractory HGOS patients, have been considered. This review provides an overview of immunotherapeutic treatments targeting, counteracting or exploiting the different immune cell compartments present in the HGOS tumor microenvironment. The strategies and possible mechanisms of HGOS cells to escape these treatments are discussed, as well as ongoing immune-based trials and recent clinical study results.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Biochemistry & Molecular Biology
Eleonora Centonze, Antonella Marte, Martina Albini, Anna Rocchi, Fabrizia Cesca, Martina Chiacchiaretta, Thomas Floss, Pietro Baldelli, Stefano Ferroni, Fabio Benfenati, Pierluigi Valente
Summary: Neuron-restrictive silencer factor/repressor element 1 (NRSF/REST) is a transcriptional repressor that regulates the expression of neural genes. In astrocytes, NRSF/REST is highly expressed and plays a role in maintaining intrinsic excitability and synaptic transmission. Its deletion leads to impaired astrocytic homeostatic functions and increased neuronal excitability.
JOURNAL OF NEUROCHEMISTRY
(2023)
Review
Oncology
Lorena Landuzzi, Francesca Ruzzi, Pier-Luigi Lollini, Katia Scotlandi
Summary: Synovial sarcoma is a rare malignant tumor characterized by t(X;18) translocation encoding the SS18-SSX fusion gene, which interacts with BAF enhancer and polycomb repressor complexes to regulate gene transcription. Different experimental in vivo models for synovial sarcoma research include transgenic mouse models, patient-derived xenografts, and cell lines. These models have contributed to identifying vulnerabilities and developing new therapies for synovial sarcoma.
Article
Biology
Federica Scotto di Carlo, Sharon Russo, Francesc Muyas, Maria Mangini, Lorenza Garribba, Laura Pazzaglia, Rita Genesio, Flavia Biamonte, Anna Chiara De Luca, Stefano Santaguida, Katia Scotlandi, Isidro Cortes-Ciriano, Fernando Gianfrancesco
Summary: Profilin 1, encoded by PFN1, is a protein that plays a tumor suppressive role in various adenocarcinomas and pagetic osteosarcomas. However, its exact contribution to tumor development is not fully understood. This study shows that inactivation of Profilin 1 leads to multiple mitotic defects, resulting in chromosomal instability and genome rearrangements in pagetic osteosarcomas. Mechanistically, Profilin 1 is involved in regulating cell division and its deficiency impairs actin filament supply during cytokinesis.
COMMUNICATIONS BIOLOGY
(2023)
Article
Oncology
Riccardo Ray Colciago, Irene Fischetti, Carlotta Giandini, Eliana La Rocca, Tiziana T. Rancati, Alicia Rejas Mateo, Mario Paolo Colombo, Laura Lozza, Claudia Chiodoni, Elena Jachetti, Maria Carmen De Santis
Summary: Oncological treatments are rapidly changing with the introduction of targeted anticancer drugs and regimens, and radioimmunotherapy is emerging as a promising field. This review provides an overview of the synergistic use of radiotherapy and immunotherapy and addresses important questions about its implementation, the patient selection criteria, and when it becomes standard clinical practice.
EXPERT REVIEW OF ANTICANCER THERAPY
(2023)
Article
Biochemistry & Molecular Biology
Michele Fiore, Alberto Taddia, Valentina Indio, Salvatore Nicola Bertuccio, Daria Messelodi, Salvatore Serravalle, Jessica Bandini, Filippo Spreafico, Daniela Perotti, Paola Collini, Andrea Di Cataldo, Gianandrea Pasquinelli, Francesca Chiarini, Maura Fois, Fraia Melchionda, Andrea Pession, Annalisa Astolfi
Summary: Clear cell sarcoma of the kidney (CCSK) is a rare pediatric renal tumor with worse prognosis than Wilms' tumor. This study aims to investigate the molecular differences between metastatic and localized BCOR-ITD-positive CCSK at diagnosis. The findings suggest that metastatic CCSKs have an overexpression of genes related to the MAPK signaling pathway, and FGF3 may play a role in the acquisition of a more aggressive phenotype.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Biochemistry & Molecular Biology
Carmen Ciavarella, Ilenia Motta, Francesco Vasuri, Teresa Palumbo, Anthony Paul Lisi, Alice Costa, Annalisa Astolfi, Sabrina Valente, Piera Versura, Eugenio F. Fornasiero, Raffaella Mauro, Mauro Gargiulo, Gianandrea Pasquinelli
Summary: The failure of arteriovenous fistulas due to intimal hyperplasia is a serious problem for patients undergoing hemodialysis. This study investigated the role of the PPAR-gamma in regulating intimal hyperplasia and found that the PPAR-gamma agonist pioglitazone had a negative effect on cell proliferation and migration.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Oncology
Sara Mongiorgi, Alessia De Stefano, Stefano Ratti, Valentina Indio, Annalisa Astolfi, Irene Casalin, Andrea Pellagatti, Stefania Paolini, Sarah Parisi, Michele Cavo, Andrea Pession, James A. McCubrey, Pann-Ghill Suh, Lucia Manzoli, Jacqueline Boultwood, Carlo Finelli, Lucio Cocco, Matilde Y. Follo
Summary: This study found that high levels of miR-192-5p are associated with higher overall survival and leukemia-free survival in myelodysplastic syndromes responding to azacitidine and lenalidomide. Moreover, miR-192-5p specifically targets and inhibits BCL2, possibly regulating proliferation and apoptosis and leading to the identification of new therapeutic targets.
CLINICAL EPIGENETICS
(2023)
Review
Genetics & Heredity
Angela Schipani, Margherita Nannini, Annalisa Astolfi, Maria A. Pantaleo
Summary: Loss of function of succinate dehydrogenase complex is a characteristic of 20-40% of KIT/PDGFRA-negative GIST. Half of SDH-deficient GIST patients lack SDHx mutations and are caused by hypermethylation of SDHC promoter. The remaining 50% have mutations in SDH subunits, with SDHA mutations being the most common.
Article
Oncology
Elisabetta Setola, S. Benini, A. Righi, G. Gamberi, E. Carretta, C. Ferrari, S. Avnet, E. Palmerini, G. Magagnoli, M. Gambarotti, P. L. Lollini, M. Cesari, S. Cocchi, A. Paioli, A. Longhi, K. Scotlandi, M. A. Laginestra, D. M. Donati, N. Baldini, T. Ibrahim
Summary: This study found a higher frequency of IDH mutations in G3 CCBC compared to G2. No significant differences in overall survival, relapse-free survival, and progression-free survival were observed based on the mutational status. After relapse, a higher rate of G3 was observed in IDH mutated CCBC.
Review
Biochemistry & Molecular Biology
Alice Costa, Livia Gozzellino, Margherita Nannini, Annalisa Astolfi, Maria Abbondanza Pantaleo, Gianandrea Pasquinelli
Summary: Visceral sarcomas are a rare subtype of soft tissue sarcomas, and understanding their development mechanisms and developing personalized therapies are urgent clinical needs. However, the complex genomic profiles and lack of reliable preclinical models present challenges.
Review
Biochemistry & Molecular Biology
Caterina Mancarella, Andrea Morrione, Katia Scotlandi
Summary: Sarcomas are a common type of bone and soft tissue cancers in children and adolescents. Limited therapeutic options exist for these cancers, but targeted protein degradation (TPD) shows promise in overcoming some of the challenges in sarcoma treatment.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)