← Back to all signals
RESEARCH PAPER ANALYSIS

Core transcriptional regulatory circuitry identified from patient-derived tumor cells provides a specific vulnerability for rhabdomyosarcoma.

AI interpretation is pending for this paper.

Open original publication →
PMID42288534
JournalCell death & disease
Publication Date2026-06-13
Ingested2026-08-02 12:07 AM
EXECUTIVE SUMMARY

What the AI sees

Not AI summarized yet.

WHY IT MATTERS

Research significance

Pending deeper interpretation.

ABSTRACT

Source abstract

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children and adolescents, with limited targeted treatment options. Core transcriptional regulatory circuitry (CRC) proteins play crucial roles in tumor cell identity and survival. Here, we established 15 patient-derived tumor cells (PDCs) from RMS specimens and found that these cells retained the expression of RMS hallmark and dependency genes, as well as the genetic mutations in primary tumors. Cleavage under targets and tagmentation sequencing (CUT&Tag-seq) for H3K27ac in these RMS PDCs identified super-enhancers and the associated CRC, which contained SMAD3, RUNX2, JUN and FOS. These factors formed an interconnected auto-regulatory loop that maintained the malignant cell state in RMS. Knocking down each of these genes decreased the expression of all the CRC members and impaired tumor cell growth in RMS. CDK7 inhibition by THZ1 disrupted the CRC and effectively suppressed cell proliferation in RMS. Our study demonstrates a common CRC in RMS that is essential for tumor maintenance, providing a promising therapeutic vulnerability for RMS.

SUPPORTING PAPER SET

32 more papers to review

Ranked by current scoring engine
1 The Induction Regimen Does Not Affect Stem-Cell Collection in Adult Patients with Ewing Sarcoma. Transfusion medicine and hemotherapy : offizielles Organ der Deutschen Gesellschaft fur Transfusionsmedizin und Immunhamatologie 61.6 2 Pre-clinical validation of OSCAR-3 mRNA-based CAR T cells for targeting osteosarcomas. Cytotherapy 57.3 3 Self-collected cervical samples for detection of high-risk human papillomavirus: A community outreach approach in a remote region. The Indian journal of medical research 44.0 4 [Clinical analysis of nine pediatric cases of acute myeloid leukemia with NUP98 rearrangement]. Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi 69.7 5 Parenting concerns among patients with advanced early-onset lung cancer: a qualitative study based on the ABC-X model. Frontiers in psychology 61.4 6 A cancer-predisposing syndrome is always there: a sequential occurrence of acute lymphoblastic leukemia after acute myeloid leukemia in a child with down syndrome. Frontiers in oncology 55.6 7 Ameloblastic carcinoma with isolated supramandibular lymph node metastasis in an adolescent: a case report and review of the literature. Frontiers in oncology 57.8 8 Comparison of nutritional screening tools in pediatric patients undergoing hematopoietic stem cell transplantation. Frontiers in pediatrics 61.5 9 Malignancies in inborn errors of immunity: a retrospective-prospective study on a large Italian cohort of pediatric and adult patients. Frontiers in immunology 75.2 10 Developmentally relevant proteins contribute to chemotherapeutic resistance in neuroblastoma. Frontiers in oncology 82.44 11 Recurrent Ventral Scapular Osteochondroma Presenting with Pseudo-winging in an Adolescent: A Case Report and Follow-up of a Previously Published Case. Journal of orthopaedic case reports 57.5 12 Angiomatoid Fibrous Histiocytoma in a Child with Hereditary Spherocytosis: A Rare Co-occurrence. Journal of orthopaedic case reports 47.5 13 Clinicopathological Spectrum and Management of Aneurysmal Bone Cysts in Atypical Anatomical Location: A Comprehensive Review of Demographics, Presentation, Diagnosis, and Treatment Outcomes. Journal of orthopaedic case reports 62.54 14 Pubertal Testicular Function in Childhood Cancer Survivors and Future Fertility. JAMA network open 72.5 15 Clinical outcomes during pregnancy and the postpartum period in women with myasthenia gravis and their newborns. Arquivos de neuro-psiquiatria 65.9 16 p53 wild-type/low c-Myc-expressing ONS76 pediatric medulloblastoma cells become senescent after gamma-irradiation and can be eliminated by senolytic substances. Cell death discovery 66.0 17 The potential role of miR-34a and miR-34c in the regulation of pediatric high-grade glioma 4 aggressiveness. Pediatric research 53.6 18 Current evidence for first-line advanced therapies in pediatric ulcerative colitis: a critical review. Inflammatory bowel diseases 65.8 19 [Clinicopathological and molecular genetic characteristics of embryonal rhabdomyosarcoma with divergent neuroectodermal differentiation: an analysis of 17 cases]. Zhonghua bing li xue za zhi = Chinese journal of pathology 58.5 20 Menin inhibitors in acute leukemia: Advances and future directions. Hematology/oncology and stem cell therapy 88.96 21 Predictors of postdischarge mortality among hospitalised children aged 5-14 years at tertiary hospitals in Tanzania: a prospective observational cohort study. BMJ paediatrics open 78.2 22 Identifying Patterns of Hospitalizations Among Childhood Cancer Survivors: A Population-Based Study. Pediatric blood & cancer 65.0 23 Re: Schreck et al. - Critical considerations for BRAF-altered tumors in children. Neuro-oncology 47.5 24 Timing of radiotherapy and autologous stem cell transplantation in embryonal tumor with multilayered rosettes: a critical narrative review. Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery 73.64 25 A phase 2 study of tipifarnib in children and young adults with tumors harboring HRAS genomic alterations: NCI-COG Pediatric MATCH trial (APEC1621M). The oncologist 86.8 26 Neonatal Dried Blood Spot Metabolomics Reveals Signatures of Early-Onset Myeloproliferative Neoplasms. International journal of cancer 60.5 27 Ultrathin endoscope-assisted pancreatobiliary interventions: from direct transpapillary access to created access routes. Digestion 61.1 28 What Is the Natural History and Likelihood of Progression of Indeterminate Pulmonary Nodules in Patients With Extremity Osteosarcoma? Clinical orthopaedics and related research 65.1 29 Outcomes of sequential keratoplasty for bilateral gelatinous drop-like corneal dystrophy: A case report. Medicine 53.4 30 Giant cystic lymphangioma in the pancreatic tail-splenic hilum region managed by en bloc resection: A case report. Medicine 53.4 31 Multidimensional Evaluation of the Efficacy of a Comprehensive Ayurveda Approach as an Add-On to Conventional Therapy in Children With Autism Spectrum Disorder: Protocol for a Randomized, Open-Label, Parallel-Group, Active-Controlled Clinical Trial. JMIR research protocols 83.84 32 Urolithiasis in Pediatric Acute Lymphoblastic Leukemia and Lymphoblastic Lymphoma (ALL/LLy): A Single-Center Case Series of Clinical, Metabolic, and Imaging Features. Journal of pediatric hematology/oncology 59.4
PATIENT-FRIENDLY SUMMARY

Core transcriptional regulatory circuitry identified from patient-derived tumor cells provides a specific vulnerability for rhabdomyosarcoma.

For education only—not personal medical advice.

Before you continue

AI-assisted research information

Neurocompute uses AI to summarize scientific papers, interpret research signals, and suggest relevant reference links. AI-generated content can be incomplete, misleading, or wrong, and generated links may be irrelevant or unavailable.

Our reviewed outputs have performed strongly to date, but past accuracy is not a guarantee. Verify summaries, scores, claims, and links against the original publication before relying on them.

This platform is for research and education only. It does not provide medical advice, diagnosis, treatment recommendations, or clinical guidance.

Pediatric cancer research intelligence graphic
PEDIATRIC CANCER VISUAL SYSTEM

Open the Research Intelligence Map

Explore the active pediatric oncology analysis view.

Expand Intelligence View →
Full Pediatric cancer research intelligence graphic