Last update 01 Nov 2024

NAB2

Last update 01 Nov 2024

Basic Info

Synonyms

EGR-1-binding protein 2, MADER, Melanoma-associated delayed early response protein

+ [4]

Introduction

Acts as a transcriptional repressor for zinc finger transcription factors EGR1 and EGR2. Isoform 2 lacks repression ability (By similarity).

Drugs associated with NAB2

Targeting NAB2–STAT6 (University of Texas at Dallas)

Target

NAB2 x STAT6

Mechanism

NAB2 inhibitors [+1]

Active Org.

The University of Texas at Dallas

Originator Org.

The University of Texas at Dallas

Active Indication

Neoplasms

Inactive Indication-

Drug Highest PhasePreclinical

First Approval Ctry. / Loc.-

First Approval Date20 Jan 1800

100 Clinical Results associated with NAB2

100 Translational Medicine associated with NAB2

0 Patents (Medical) associated with NAB2

399

Literatures (Medical) associated with NAB2

01 Nov 2024·Brain Pathology

NAB2::STAT6 fusions and genome‐wide DNA methylation profiling: Predictors of patient outcomes in meningeal solitary fibrous tumors

Article

Author: Santagata, Sandro ; Stoehr, Robert ; Orr, Brent A. ; Brastianos, Priscilla K. ; Fritchie, Karen ; Guajardo, Andrew ; Tran, Quynh T. ; Link, Michael J. ; Moskalev, Evgeny A. ; Haller, Florian ; Nguyen, Minh P. ; Gross, John ; Eschbacher, Kathryn L. ; Perry, Arie ; Louis, David N. ; Ferrarese, Fabio ; Wu, Ashley ; Raleigh, David R. ; Brown, Paul D. ; Rossi, Sabrina ; Brat, Daniel J. ; Alexander, Brian ; Caron, Alissa ; Velazquez Vega, Jose ; Giannini, Caterina ; Jenkins, Sarah ; Kaplan, Alexander B. ; Rodriguez, Fausto ; Martinez‐Lage, Maria

AbstractMeningeal solitary fibrous tumors (SFT) are rare and have a high frequency of local recurrence and distant metastasis. In a cohort of 126 patients (57 female, 69 male; mean age at surgery 53.0 years) with pathologically confirmed meningeal SFTs with extended clinical follow‐up (median 9.9 years; range 15 days–43 years), we performed extensive molecular characterization including genome‐wide DNA methylation profiling (n = 80) and targeted TERT promoter mutation testing (n = 98). Associations were examined with NAB2::STAT6 fusion status (n = 101 cases; 51 = ex5‐7::ex16‐17, 26 = ex4::ex2‐3; 12 = ex2‐3::exANY/other and 12 = no fusion) and placed in the context of 2021 Central Nervous System (CNS) WHO grade. NAB2::STAT6 fusion breakpoints (fusion type) were significantly associated with metastasis‐free survival (MFS) (p = 0.03) and, on multivariate analysis, disease‐specific survival (DSS) when adjusting for CNS WHO grade (p = 0.03). DNA methylation profiling revealed three distinct clusters: Cluster 1 (n = 38), Cluster 2 (n = 22), and Cluster 3 (n = 20). Methylation clusters were significantly associated with fusion type (p < 0.001), with Cluster 2 harboring ex4::ex2‐3 fusion in 16 (of 20; 80.0%), nearly all TERT promoter mutations (7 of 8; 87.5%), and predominantly an “SFT” histologic phenotype (15 of 22; 68.2%). Clusters 1 and 3 were less distinct, both dominated by tumors having ex5‐7::ex16‐17 fusion (respectively, 25 of 33; 75.8%, and 12 of 18; 66.7%) and with variable histological phenotypes. Methylation clusters were significantly associated with MFS (p = 0.027), but not overall survival (OS). In summary, NAB2::STAT6 fusion type was significantly associated with MFS and DSS, suggesting that tumors with an ex5::ex16‐17 fusion may have inferior patient outcomes. Methylation clusters were significantly associated with fusion type, TERT promoter mutation status, histologic phenotype, and MFS.

10 Oct 2024·Archives of Pathology & Laboratory Medicine

High-Throughput Hybridization Assay as First-Line Diagnostic Test for Sarcomas: Clinical Assessment in a Tertiary Referral Center

Article

Author: Vargas-Padilla, María Victoria ; Delgado-Bellido, Daniel ; de Álava, Enrique ; Civantos, Gema ; Beltrán-Povea, Amparo ; Salguero-Aranda, Carmen ; Díaz-Martín, Juan ; Perez, Marco ; Marcilla, David

Context.—Sarcomas are rare and highly heterogeneous mesenchymal tumors with deceptive morphologic features that pose a challenge for precise diagnostics. Chromosomal rearrangements generating pathognomonic gene fusions are useful diagnostic markers, traditionally tested using single-plex standard of care assays with limited diagnostic yield. NanoString nCounter technology has emerged as a robust solution with multiplexing capabilities.Objective.—To optimize NanoString effective coverage of specific entities and conduct a validation study to support its clinical implementation.Design.—We reconfigured a NanoString’s codeset by including a set of probes for detecting gene fusion variants of solitary fibrous tumors, low-grade fibromyxoid sarcomas/sclerosing epithelioid fibrosarcomas, and undifferentiated small round cell sarcomas, totaling 188 probes. A technical validation study was conducted with 96 retrospective samples. Additionally, 76 prospective samples were evaluated to assess the assay’s clinical performance.Results.—Both technical and clinical validation studies showed that NanoString’s codeset reached >88% sensitivity and 100% specificity, compared with standard of care methods, and superior diagnostic yield as a first-line test. Our design enabled the detection of almost all fusion variants of NGFI-A binding protein 2 (NAB2) with signal transducer and activator of transcription 6 (STAT6) in solitary fibrous tumors, as well as cAMP responsive element binding protein 3 like 1/2 (CREB3L1/2) rearrangements in all low-grade fibromyxoid sarcoma/sclerosing epithelioid fibrosarcoma cases. Identification of specific gene fusions of undifferentiated small round cell sarcoma was also improved, but additional strategies are necessary to attain full coverage.Conclusions.—The NanoString platform demonstrated good sensitivity, specificity, and superior diagnostic yield. It is a cost-effective assay with rapid turnaround time, low sample consumption, streamlined analysis, and easy customization. Therefore, it is a promising alternative first-line diagnostic tool for routine sarcoma testing.

01 Oct 2024·Asian Journal of Endoscopic Surgery

A case of robotic distal pancreatectomy for solitary fibrous tumor of the pancreas

Article

Author: Nagata, Rihito ; Kimura, Akifumi ; Takemura, Nobuyuki ; Matsudaira, Shinichi ; Yamada, Naganori ; Imada, Hiroki ; Ninomiya, Riki ; Maki, Akira ; Beck, Yoshifumi ; Takayanagi, Natsuko

AbstractSolitary fibrous tumor (SFT) is a spindle cell tumor driven by the NAB2‐STAT6 fusion gene. While it can originate from any soft tissue, primary SFT of the pancreas is rare with limited reports. A 36‐year‐old man came to our department due to abdominal pain. Computed tomography revealed a circular mass with weak peripheral enhancement and an internal cyst in the pancreatic tail. Diagnosis was not confirmed through endoscopic ultrasound‐guided biopsy, and differential diagnoses included acinar cell carcinoma and pancreatic neuroendocrine tumor. A robotic distal pancreatectomy with splenectomy was performed, and the patient was discharged 11 days postoperatively. Histopathological examination showed an irregular arrangement of spindle cells, and immunohistochemical staining was positive for CD34 and STAT6, confirming an SFT diagnosis with low metastatic risk. Robotic surgery effectively managed this tumor.

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NAB2

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