Transcriptional regulation of KCNA2 coding Kv1.2 by EWS::FLI1: involvement in controlling the YAP/Hippo signalling pathway and cell proliferation DOI Creative Commons

Maryne Dupuy,

Anaïs Postec,

Mathilde Mullard

и другие.

Cell Communication and Signaling, Год журнала: 2024, Номер 22(1)

Опубликована: Дек. 18, 2024

Ewing sarcoma (ES), the second main pediatric bone sarcoma, is characterised by a chromosomal translocation leading to formation of fusion proteins like EWS::FLI1. While several studies have shown that potassium channels drive development many tumours, limited data exist on ES. This work therefore aimed study transcriptional regulation KCNA2 and define involvement Kv1.2 channel encoded in key function ES development, cell proliferation. expression patients lines was measured via bioinformatic analysis (RNA-Seq). The presence functional using patch-clamp experiments. Molecular biology approaches were used after EWS::FLI1 silencing KCNA2. Proliferation count assessment performed approaches. (siRNA) RNA-Seq identify signalling pathways involved ability Hippo pathway studied measuring Hippo/YAP target genes expression, while YAP protein with Western-Blot immunofluorescence research identified (encoding for channel) as highly expressed biopsies lines. results indicated correlation between patient survival. also demonstrated KCNA2/Kv1.2 direct EWS::FLI1, molecular mechanisms which this chimeric regulates gene at level. Furthermore, activity regulate proliferation drives pathway. Using specific inhibitor (κ-Conotoxin), suggested two complementary are process, both dependently independently plasma membrane. first describe cancer development. unveiled cascade. Thus, suggests could be potential therapeutic

Язык: Английский

Superficial Neurocristic EWSR1::FLI1 Fusion Tumor: A Distinctive, Clinically Indolent, S100 Protein/SOX10-Positive Neoplasm DOI
Andrew L. Folpe, Michael T. Tetzlaff,

Steven D Billings

и другие.

Modern Pathology, Год журнала: 2024, Номер 37(8), С. 100537 - 100537

Опубликована: Июнь 12, 2024

Язык: Английский

Процитировано

7

The RNA tether model for human chromosomal translocation fragile zones DOI
D Liu, Chih‐Lin Hsieh, Michael R. Lieber

и другие.

Trends in Biochemical Sciences, Год журнала: 2024, Номер 49(5), С. 391 - 400

Опубликована: Март 14, 2024

Язык: Английский

Процитировано

3

Novel classification system and high-risk categories of pediatric acute myeloid leukemia DOI Creative Commons

Masayuki Umeda,

Yen‐Chun Liu, Seth E. Karol

и другие.

Haematologica, Год журнала: 2025, Номер unknown

Опубликована: Янв. 9, 2025

The prognosis of pediatric acute myeloid leukemia (AML) remains poor compared with lymphoblastic (ALL); accurate diagnosis and treatment strategies based on the genomic background are strongly needed. Recent advances in sequencing technologies have identified novel AML subtypes, including BCL11B structural variants UBTF tandem duplications (UBTF-TD), associated prognosis. In contrast, these subtypes do not fit into diagnostic systems for 5th edition WHO classification or International Consensus Classifications (ICC) released 2022. this review, we describe current state context a new framework findings updated profiling. Molecular categories system unique transcriptional, mutational, clinical characteristics, which can be leveraged predicting outcomes developing molecular-target therapies initiating driver alterations. We also highlight four high-risk AML, namely CBFA2T3::GLIS2, BCL11B, UBTF-TD, ETS family fusions, focusing their disease mechanisms, associations, possible therapeutic to overcome dismal

Язык: Английский

Процитировано

0

Perinatal exposure to ambient fine particle air pollution and risk of childhood ewing sarcoma in a population-based case-control study in California (1988–2015) DOI Creative Commons
Cassandra J. Clark, Rong Wang, Joseph L. Wiemels

и другие.

Environmental Health, Год журнала: 2025, Номер 24(1)

Опубликована: Март 7, 2025

Abstract Background Incidence of childhood Ewing sarcoma, a rare cancer affecting bones and soft tissues, is increasing. Environmental exposures during the perinatal period, like air pollution, may play role. We examined exposure to ambient fine particulate matter (PM 2.5 ) sarcoma risk in case-control linkage study nested within California birth cohort. Methods The included 388 children born (1982–2015) diagnosed with at age 0–19 years (1988–2015), 19,341 California-born cancer-free controls frequency-matched cases on year (50:1 ratio). Ambient PM concentrations maternal residence were averaged separately over two time periods, gestation first after birth, using validated ensemble-based model (categorized as quartiles). estimated odds ratios (ORs) 95% confidence intervals (CIs) for association between risk, adjusting sex, year, race, ethnicity, weight, education stratifying by Hispanic ethnicity assess potential disparities -related risk. Results In overall population, was not associated when considering or birth. Among children, who experienced greater pollution compared non-Hispanic higher levels yielded elevated quartile (Q2 OR [95% CI] = 1.53 [0.94–2.51]; Q3 1.56 [0.95–2.56]; Q4 1.39 [0.79–2.47]). also relation Conclusion Our results provide new suggestive evidence that contribute although these findings statistically significant specific children. These require replication underscore need further evaluate role -cancer relationship genetic ancestry measures through lens environmental justice.

Язык: Английский

Процитировано

0

The Double Life of microRNAs in Bone Sarcomas: Oncogenic Drivers and Tumor Suppressors DOI Open Access
Stefano Zoroddu,

Fabio Sias,

Luigi Bagella

и другие.

International Journal of Molecular Sciences, Год журнала: 2025, Номер 26(10), С. 4814 - 4814

Опубликована: Май 17, 2025

Bone sarcomas, including Osteosarcoma, Ewing’s sarcoma, and Chondrosarcoma, are rare yet aggressive tumors with high metastatic potential poor survival outcomes. Despite advances in surgical chemotherapeutic techniques, these malignancies remain difficult to treat. They often exhibit resistance conventional therapies associated a limited prognosis for patients. MicroRNAs (miRNAs) have emerged as pivotal regulators of cancer biology, orchestrating crucial processes such cell proliferation, apoptosis, metastasis. Their double life oncogenes or tumor suppressors underscores their significance the pathogenesis bone sarcomas. This review examines multifaceted roles miRNAs malignancies. By elucidating complex networks affected by miRNA dysregulation, we seek identify novel avenues miRNA-based interventions. It is intention this work stimulate future research clinical strategies that exploit transform management outcomes

Язык: Английский

Процитировано

0

Extra-skeletal Ewing sarcoma in a 63-year-old female with a history of triple-negative breast cancer: a case report and literature review DOI Creative Commons
Ming‐sho Ho,

Golnesa Safavi,

Won Jin Jeon

и другие.

Frontiers in Oncology, Год журнала: 2025, Номер 15

Опубликована: Май 23, 2025

Ewing sarcoma (ES), particularly Extra-skeletal (EES), is a rare, aggressive tumor predominantly affecting adolescents and young adults, yet it can occur in older patients, leading to misdiagnosis delay treatment. The standard approach includes surgical resection, chemotherapy, radiation therapy for unresectable disease. This case report presents 63-year-old female patient with history of triple-negative breast cancer, who was discovered have soft tissue the left medial thigh. Initially misdiagnosed as rhabdomyosarcoma, diagnosis EES ultimately confirmed via RNA sequencing revealing EWSR1-FLI1 fusion gene. She underwent neoadjuvant chemotherapy followed by radical resection tumor.

Язык: Английский

Процитировано

0

Bone cancer: Biomarker landscape DOI
S. Sonia, Muhammad Irfan, Ameer Fawad Zahoor

и другие.

Elsevier eBooks, Год журнала: 2025, Номер unknown, С. 75 - 109

Опубликована: Янв. 1, 2025

Язык: Английский

Процитировано

0

Preclinical models for the study of pediatric solid tumors: focus on bone sarcomas DOI Creative Commons
D. Isabel Petrescu, Jason T. Yustein, Atreyi Dasgupta

и другие.

Frontiers in Oncology, Год журнала: 2024, Номер 14

Опубликована: Июль 18, 2024

Sarcomas comprise between 10–15% of all pediatric malignancies. Osteosarcoma and Ewing sarcoma are the two most common bone tumors diagnosed in children young adults. These commonly treated with surgery and/or radiation therapy combination chemotherapy. However, there is a strong need for development utilization targeted therapeutic methods to improve patient outcomes. Towards accomplishing this goal, pre-clinical models these unique malignancies particular importance design test experimental strategies prior being introduced patients due their origination site propensity metastasize. Pre-clinical offer several advantages study sarcomas benefits shortcomings dependent on type model. This review addresses types available solid tumors, special attention osteosarcoma sarcoma.

Язык: Английский

Процитировано

2

Signaling pathways and targeted therapies in Ewing sarcoma DOI Creative Commons
Jia Ke, Li Cao, Yihan Yu

и другие.

Pharmacology & Therapeutics, Год журнала: 2024, Номер 266, С. 108765 - 108765

Опубликована: Ноя. 30, 2024

Язык: Английский

Процитировано

2

Bioinformatics Analysis of the Biological Properties of Ewing Sarcoma DOI Open Access

Luchang Chen,

Huifang Zeng,

Wujia Yang

и другие.

Journal of Biosciences and Medicines, Год журнала: 2024, Номер 12(06), С. 202 - 215

Опубликована: Янв. 1, 2024

Язык: Английский

Процитировано

0