NKX2-2

Last update 23 Jan 2025

Basic Info

Synonyms

Homeobox protein NK-2 homolog B, Homeobox protein Nkx-2.2, NK2 homeobox 2

+ [3]

Introduction

Transcriptional activator involved in the development of insulin-producting beta cells in the endocrine pancreas (By similarity). May also be involved in specifying diencephalic neuromeric boundaries, and in controlling the expression of genes that play a role in axonal guidance. Binds to elements within the NEUROD1 promoter (By similarity).

100 Clinical Results associated with NKX2-2

100 Translational Medicine associated with NKX2-2

0 Patents (Medical) associated with NKX2-2

868

Literatures (Medical) associated with NKX2-2

01 Feb 2025·Modern Pathology

Superficial Neurocristic FET::ETS Fusion Tumor: Expanding the Clinicopathological and Molecular Genetic Spectrum of a Recently Described Entity

Article

Author: Kerr, Darcy A. ; Folpe, Andrew L ; Warmke, Laura M. ; Dehner, Carina A ; Cloutier, Jeffrey M ; Ameline, Baptiste ; Billings, Steven D ; Cloutier, Jeffrey M. ; Que, Syril Keena T ; Dehner, Carina A. ; Fritz, Mike ; Umphress, Brandon ; Kerr, Darcy A ; Dermawan, Josephine K. ; Malik, Faizan ; Fritchie, Karen J. ; Billings, Steven D. ; Linos, Konstantinos ; Baumhoer, Daniel ; Fritchie, Karen J ; Folpe, Andrew L. ; Que, Syril Keena T. ; Dermawan, Josephine K ; Warmke, Laura M

Superficial neurocristic EWSR1::FLI1 fusion tumor is a very recently described, clinically indolent tumor of the skin and superficial soft tissues, which differs in essentially all ways from Ewing sarcoma, despite harboring an identical fusion event. The EWSR1 and FLI1 genes are members of the FET and ETS gene family, respectively, and very rare examples of Ewing sarcoma harbor alternative FET::ETS fusion events, such as EWSR1::ERG, FUS::FLI1, FUS::ERG, EWSR1::ETV4, and others. We report 5 new cases of this very rare entity, harboring in 3 cases alternative FET::ETS fusion events. The tumors occurred in 2 males and 3 females (median age, 14 years, range, 8-69 years) and presented as solitary dermal/subcutaneous masses of the thigh, foot, shoulder, arm, and back (median size, 1.8 cm; range, 1-2 cm). All patients underwent wide excisions; one received adjuvant chemotherapy. Clinical follow-up on 3 patients (median, 24 months; range, 18-31 months) showed all to be without disease. Morphologically, all tumors displayed typical features of this entity as described, with nests of cytologically bland, diffusely S100 protein/SOX10-positive round cells without mitotic activity, surrounded by fibrous bands containing spindled cells with similar nuclear features. The tumors also showed membranous CD99 (4/5) and nuclear NKX2.2 (3/3) expression. RNA sequencing (5 cases) demonstrated FUS::FLI1, FUS::ERG, EWSR1::FLI1, EWSR1::ERG, and a novel FUS::ETV5. Methylation profiling (4 cases) showed all to cluster with previously reported superficial neurocristic EWSR1::FLI1 fusion tumors and apart from conventional and "adamantinoma-like" Ewing sarcoma. Our findings confirm the distinctive clinicopathological features of this very rare, recently described entity and expand its molecular genetic spectrum. Reflecting on these findings, we propose modifying the name of this entity to "superficial neurocristic FET::ETS fusion tumor."

01 Jan 2025·Archives of Pathology & Laboratory Medicine

Clinicopathologic Correlates of PIT1 and SF1-Multilineage Pituitary Neuroendocrine Tumors and the Diagnostic Utility of NKX2.2 Immunohistochemistry in Pituitary Pathology

Article

Author: Erkan, Buruç ; Karatay, Hüseyin ; Burhan, Şebnem ; Yılmaz-Özgüven, Banu ; Doğukan, Fatih Mert ; Yüzkan, Sabahattin

Context.—PIT1 and SF1-multilineage pituitary neuroendocrine tumors (PitNETs) have been defined since the classification of adenohypophysial tumors based on the PIT1, SF1, and TPIT transcription factors.Objective.—To describe the clinicopathologic features of PIT1 and SF1-multilineage PitNETs and to contribute to the pituitary pathology practice by questioning the expression of NKX2.2 in PitNETs.Design.—We reviewed 345 PitNETs and described the clinicopathologic features of 8 PIT1 and SF1-multilineage tumors. NKX2.2 positivity and staining pattern were compared to those of 45 PitNETs from the control group.Results.—PIT1 and SF1-multilineage PitNET patients had a mean age of 41.13 (range, 14–58 years) and a mean tumor diameter of 14.0 mm (range, 8–20 mm). The most common clinical presentation was acromegaly (6 of 8), and postoperative remission was achieved in all patients. On histomorphologic examination, a pseudopapillary pattern was seen in 5 of the tumors, either focally or diffusely. In addition to PIT1 and SF1, there was a diffuse staining with growth hormone and a predominantly perinuclear staining with cytokeratin 18. With NKX2.2, all multilineage tumors were positive, of which 5 were diffuse and 3 were focal. In the control group, 8 tumors (8 of 45) were positive, of which only 1 was diffuse and 7 were focal.Conclusions.—In conclusion, NKX2.2 is a transcription factor that can be used as an additional tool in pituitary pathology, and PIT1 and SF1-multilineage PitNETs are specific tumors that usually present with acromegaly, show signs of a nonaggressive clinical course, have a pseudopapillary histomorphology, and express NKX2.2.

13 Dec 2024·Science Advances

Nzf2 promotes oligodendrocyte differentiation and regeneration via repressing HDAC1-mediated histone deacetylation

Article

Author: Dai, Zhong-Min ; Xu, Xiaofeng ; Huang, Hao ; Yang, Junlin ; Chen, Lixia ; Qiu, Mengsheng ; Fang, Minxi

Proper axonal myelination and function of the vertebrate central nervous system rely largely on the timely differentiation of oligodendrocytes (OLs), yet key regulatory factors remain enigmatic. Our study reveals neural zinc finger (Nzf2) as a crucial orchestrator that controls the timing of OL differentiation both during development and myelin repair, contrasting with its previously suggested role in direct myelin gene regulation. Nzf2 ablation delays the onset of OL differentiation, while hyperactivation stimulates OL differentiation both during development and remyelination. Using RNA-seq and ChIP-seq, we pinpoint Nkx2.2 as a critical downstream target of Nzf2. Specific binding of Nzf2 in the Nkx2.2 gene locus inhibits histone deacetylation by disrupting the HDAC1 repressor complex and reducing deacetylase activity. Furthermore, Nzf2 overrides the inhibitory Notch signaling to initiate OL differentiation. Thus, we propose that the Notch-Nzf2-Nkx2.2 axis is a vital component of OL differentiation timing mechanism, suggesting Nzf2 as a potential therapeutic target for stimulating OL differentiation and boosting myelin repair in demyelinating diseases.

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