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Last update 08 May 2025

USP6

Last update 08 May 2025

Basic Info

Synonyms

Deubiquitinating enzyme 6, HRP1, Proto-oncogene TRE-2

+ [9]

Introduction

Deubiquitinase with an ATP-independent isopeptidase activity, cleaving at the C-terminus of the ubiquitin moiety. Catalyzes its own deubiquitination. In vitro, isoform 2, but not isoform 3, shows deubiquitinating activity. Promotes plasma membrane localization of ARF6 and selectively regulates ARF6-dependent endocytic protein trafficking. Is able to initiate tumorigenesis by inducing the production of matrix metalloproteinases following NF-kappa-B activation. May act as a GTPase-activating protein for RAB3A (PubMed:19077034).

Drugs associated with USP6

MER-101 (Merlin)

Target

USP6

Mechanism

USP6 modulators

Active Org.

MERLIN Biotech LLC

Originator Org.

MERLIN Biotech LLC

Active Indication

Sarcoma [+1]

Inactive Indication-

Drug Highest PhasePreclinical

First Approval Ctry. / Loc.-

First Approval Date20 Jan 1800

100 Clinical Results associated with USP6

100 Translational Medicine associated with USP6

0 Patents (Medical) associated with USP6

374

Literatures (Medical) associated with USP6

01 Mar 2025·In Vivo

Fibroma of Tendon Sheath Revisited

Review

Author: Nakayama, Shizuhide ; Aoki, Mikiko ; Koga, Takamasa ; Shinohara, Yuki ; Nishio, Jun ; Koga, Mikoro

Fibroma of tendon sheath (FTS) is a benign fibroblastic/myofibroblastic neoplasm that primarily occurs in the fingers and hands of young and middle-aged adults. The lesion typically presents as a small, firm, slow-growing, painless nodule. Ultrasonography usually shows a focal nodular mass with homogeneous hypoechogenicity. Magnetic resonance imaging reveals a well-defined nodular mass with decreased signal on all pulse sequences. No or minimal peripheral enhancement is often seen after intravenous contrast. Histologically, the lesion is well circumscribed and consists of bland spindle cells in a dense collagenous stroma with slit-like thin-walled vessels at the periphery. A cellular variant of FTS has also been described and shows at least a focal morphological overlap with nodular fasciitis. Immunohistochemistry does not play a significant role in the diagnosis of FTS. Cytogenetic studies have demonstrated the presence of 11q rearrangements. A significant subset of cellular variants of FTS are characterized by ubiquitin specific peptidase 6 (USP6) rearrangements, with a variety of fusion partners. Complete surgical excision is the treatment of choice. This review provides an updated overview of the clinical, radiological, histological, cytogenetic and molecular genetic features of FTS and discusses the differential diagnosis of this uncommon entity.

01 Feb 2025·BMJ Case Reports

Complete remodelling post-intralesional resection of an aggressive proximal humerus aneurysmal bone cyst mimicking telangiectatic osteosarcoma

Article

Author: Singh, Harpreet ; Mahadev, Arjandas ; Zainuddin, Mohammad Ashik Bin ; Wong, Kenneth Pak Leung ; Aw, Sze Jet

We describe a case of an aggressive proximal humerus aneurysmal bone cyst (ABC) mimicking telangiectatic osteosarcoma with complete remodelling and reconstitution of the bone. A boy in middle childhood presented with a pathological fracture preceded by progressive right shoulder pain and swelling. Imaging and open biopsy supported the diagnosis of primary ABC with positive ubiquitin-specific protease 6 gene fusion. Treatment was initially refused, leading to significant bone erosion. Subsequently, intralesional resection and grafting were performed, confirming the ABC diagnosis. Three years post-surgery, radiographs show excellent bone remodelling, with the child maintaining premorbid shoulder function. By performing an intralesional resection, there was no doubt of tissue representation, and it allows contamination to be minimised should a wide resection be necessary later. Interestingly, there was complete reconstitution and remodelling of the proximal humerus; likely from intact periosteum like that seen in the donor sites of fibula strut grafts.

01 Jan 2025·International Dental Journal

Aneurysmal Bone Cyst of the Jaws: A Retrospective Analysis of 19 Cases

Article

Author: Zhang, Chunye ; Han, Jing ; Liu, Jiannan ; Zhang, Yiwen

OBJECTIVETo analyse the clinical, radiographic, and pathological characteristics of aneurysmal bone cyst of the jaws (JABCs), and to provide reference for clinical diagnosis and treatment.MATERIALS AND METHODSA retrospective study was conducted on 19 patients with JABC diagnosed from 2018 to 2022.RESULTSOf the 19 JABC patients (5 males, 14 females, aged 7-53 years, mean 23.7), 94.7% of the lesions were in the mandible, with only 5.3% in the maxilla. Painless swelling was the most common symptom. CT typically showed bone expansion with either a unilocular or multilocular appearance and visible intracystic septa. MRI revealed characteristic 'fluid-fluid' levels within the lesion. Microscopically, most cases were unilocular or multilocular; 10.5% were cystic-solid, and USP6 gene disruption was detected in 44.4% of tested cases (4/9). All the cases were treated surgically, ranging from curettage to extensive excision. The follow-up data of 16 patients were obtained, most of them had a good prognosis, with one patient relapsed 9 months after surgery.CONCLUSIONJABC commonly occurs in the mandible, with a higher incidence in women. Swelling is the most common symptom. Radiographic findings were mostly unilocular or multilocular lesions with clear boundaries, and the characteristic multiple 'fluid-fluid' level could be used as an important basis for diagnosis. Smaller lesions can achieve good therapeutic effects through curettage, while larger ones often require radical excision followed by autologous bone graft reconstruction simultaneously. Prognosis is favourable in most cases.CLINICAL RELEVANCEJABC are rare but clinically significant, requiring accurate diagnosis and timely treatment. This study analyses 19 cases, highlighting their diverse clinical, imaging, and histological features. Early detection and personalized treatment, including surgery, are crucial to prevent recurrence and preserve function. Long-term follow-up is essential for monitoring outcomes. These findings enhance the understanding of JABC, guiding clinicians in management for better patient outcomes.

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