ArticleOncogene2010
TRE17/USP6 oncogene translocated in aneurysmal bone cyst induces matrix metalloproteinase production via activation of NF-kappaB.
Article in Oncogene, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 72 papers.
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Who cites it
72 citing papers in PubMed, 152 citations in OpenAlex.
- Primary malignant aneurysmal bone cyst of the metatarsal with PAFAH1B1::USP6 fusion: expanding the clinicopathologic spectrum of malignant USP6 translocated neoplasms.Skeletal radiology · 2026Article
- A rare calvarial aneurysmal bone cyst in an adult: Case report and literature review.Surgical neurology international · 2026Article
- Intracranial aneurysmal bone cysts in association with fibrous dysplasia: Case report and scoping review of case reports.Surgical neurology international · 2026Article
- The role of USP19 in human diseases: from molecular function to clinical relevance.Frontiers in immunology · 2026Review
- Rethinking the role of bisphosphonates after denosumab treatment in locally advanced or unresectable aneurysmal bone cysts: A meta-analysis.World journal of orthopedics · 2025Article
- Physiological roles and therapeutic implications of USP6.Cell death discovery · 2025Review
- Aneurysmal Bone Cyst of the Distal Ulna in an Elderly Lady.Journal of orthopaedic case reports · 2024Article
- Ipsilateral Fibula Grafting in a Case of Aneurysmal Bone Cyst in Tibia Diaphysis: A Case Report.Journal of orthopaedic case reports · 2024Article
- Article
- Management of aggressive recurrent thoracic spine aneurysmal bone cyst in a 7-year-old male: A case report and review of the literature.Surgical neurology international · 2024Article
- Polidocanol sclerotherapy for the treatment of aneurysmal bone cyst, with or without pathological fractures: A prospective, comparative study.Journal of orthopaedics · 2023Article
- Review
- The promotive role of USP1 inhibition in coordinating osteogenic differentiation and fracture healing during nonunion.Journal of orthopaedic surgery and research · 2023Article
- Aneurysmal bone cyst: a review.Journal of pathology and translational medicine · 2023Review
- Histopathogenesis of bone- and soft-tissue tumor spectrum with USP6 gene rearrangement: multiple partners involved in the tissue repair process.Histology and histopathology · 2023Review
- Single dose intralesional doxycycline foam infusion promotes bone growth and resolution of an odontoid aneurysmal bone cyst.Surgical neurology international · 2023Article
- Benign Brain and Spinal Tumors Originating from Bone or Cartilage.Advances in experimental medicine and biology · 2023Article
- Aneurysmal bone cysts in the spine, causing neurological compromise: safety and clinical efficacy of sclerotherapy utilizing sodium Tetradecyl sulfate foam.Skeletal radiology · 2021Article
- Article
- Ubiquitin-Specific Protease 6 Functions as a Tumor Suppressor in Ewing Sarcoma through Immune Activation.Cancer research · 2021Article
12 more citing papers are in PubMed but not listed here.
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Authors and funding
11 authors at 5 institutions in 3 countries.
Funding
Abstract
Aneurysmal bone cyst (ABC) is an aggressive, pediatric bone tumor characterized by extensive destruction of the surrounding bone. Although first described over 60 years ago, its molecular etiology remains poorly understood. Recent work revealed that ABCs harbor translocation of TRE17/USP6, leading to its transcriptional upregulation. TRE17 encodes a ubiquitin-specific protease (USP), and a TBC domain that mediates binding to the Arf6 GTPase. However, the mechanisms by which TRE17 overexpression contributes to tumor pathogenesis, and the role of its USP and TBC domains, are unknown. ABCs are characterized by osteolysis, inflammatory recruitment and extensive vascularization, the processes in which matrix proteases have a prominent role. This led us to explore whether TRE17 regulates the production of matrix metalloproteinases (MMPs). In this study we show that TRE17 is sufficient to induce expression of MMP-9 and MMP-10, in a manner requiring its USP activity, but not its ability to bind Arf6. TRE17 induces transcription of MMP-9 through activation of nuclear factor-kappaB (NF-kappaB), mediated in part by the GTPase RhoA and its effector kinase, ROCK. Furthermore, xenograft studies show that TRE17 induces formation of tumors that reproduce multiple features of ABC, including a high degree of vascularization, with an essential role for the USP domain. In sum, these studies reveal that TRE17 is sufficient to initiate tumorigenesis, identify MMPs as novel TRE17 effectors that likely contribute to ABC pathogenesis and define the underlying signaling mechanism of their induction.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.