NR4A3 (nuclear receptor subfamily 4, group A, member 3)
2003-07-01 Goran Stenman   AffiliationLundberg Laboratory for Cancer Research, Department of Pathology, Goteborg University, Sahlgrenska University Hospital, SE-413 45 Goteborg, Sweden
DNA/RNA
Description
spans about 46 kb; 9 exons
Transcription
four transcript variants (6382 bp, 5635 bp, 4983bp and 2588 bp)
Proteins
Description
three isoforms (637 aa, 626 aa and 443 aa); N-terminal activation domain; central bipartite zinc finger DNA-binding domain; C-terminal ligand binding domain.
Expression
most tissues, highest expression levels in the central nervous system.
Localisation
nuclear
Function
orphan nuclear receptor; implicated in the control of cell proliferation as an immediate-early protein and in differentiation and apoptosis; binds as a monomer to the DNA-response element NBRE; activate transcription constitutively when bound to NBRE; the homeotic protein Six3 is a coactivator of TEC.
Homology
belongs to the steroid/thyroid receptor gene super family; highly homologous to the nuclear receptors NGFI-B and NURR1.
Implicated in
Disease
rare type of sarcoma (2.3% of all soft tissue sarcomas) characteristically involving the deep, soft tissues of the extremities; morphological resemblance to embryonic cartilage.
Hybrid gene
EWSR1-TEC
Fusion protein
N-terminal transactivation domain of EWSR1 fused to the entire TEC protein.
Disease
rare type of sarcoma (2.3% of all soft tissue sarcomas) characteristically involving the deep, soft tissues of the extremities; morphological resemblance to embryonic cartilage.
Hybrid gene
TAF2N-TEC
Fusion protein
N-terminal transactivation domain of TAF2N fused to the entire TEC protein.
Entity name
Extraskeletal myxoid chondrosarcoma with t(9;15)(q22;q21).
Disease
rare type of sarcoma (2.3% of all soft tissue sarcomas) characteristically involving the deep, soft tissues of the extremities; morphological resemblance to embryonic cartilage.
Hybrid gene
TCF12-TEC
Fusion protein
N-terminal domain of TCF12 fused to the entire TEC protein; the N-terminal of TCF12 shows no sequence homology to the N-terminals of EWSR1 or TAF2N.
Breakpoints

Article Bibliography
| Pubmed ID | Last Year | Title | Authors |
|---|---|---|---|
| 10602520 | 1999 | Identification of a novel fusion gene involving hTAFII68 and CHN from a t(9;17)(q22;q11.2) translocation in an extraskeletal myxoid chondrosarcoma. | Attwooll C et al |
| 8570200 | 1996 | Fusion of the EWS gene to CHN, a member of the steroid/thyroid receptor gene superfamily, in a human myxoid chondrosarcoma. | Clark J et al |
| 8614405 | 1995 | The isolation and characterization of MINOR, a novel mitogen-inducible nuclear orphan receptor. | Hedvat CV et al |
| 10359536 | 1999 | The EWS/TEC fusion protein encoded by the t(9;22) chromosomal translocation in human chondrosarcomas is a highly potent transcriptional activator. | Labelle Y et al |
| 8634690 | 1995 | Oncogenic conversion of a novel orphan nuclear receptor by chromosome translocation. | Labelle Y et al |
| 12543801 | 2003 | The homeotic protein Six3 is a coactivator of the nuclear receptor NOR-1 and a corepressor of the fusion protein EWS/NOR-1 in human extraskeletal myxoid chondrosarcomas. | Laflamme C et al |
| 12049818 | 2002 | The AF2 domain of the orphan nuclear receptor TEC is essential for the transcriptional activity of the oncogenic fusion protein EWS/TEC. | Maltais A et al |
| 7811288 | 1994 | Molecular cloning of a novel thyroid/steroid receptor superfamily gene from cultured rat neuronal cells. | Ohkura N et al |
| 11673470 | 2002 | The EWS/NOR1 fusion gene product gains a novel activity affecting pre-mRNA splicing. | Ohkura N et al |
| 11156374 | 2000 | Fusion of the NH2-terminal domain of the basic helix-loop-helix protein TCF12 to TEC in extraskeletal myxoid chondrosarcoma with translocation t(9;15)(q22;q21). | Sjögren H et al |
Other Information
Locus ID:
NCBI: 8013
MIM: 600542
HGNC: 7982
Ensembl: ENSG00000119508
Variants:
dbSNP: 8013
ClinVar: 8013
TCGA: ENSG00000119508
COSMIC: NR4A3
RNA/Proteins
Expression (GTEx)
Pathways
Protein levels (Protein atlas)
References
| Pubmed ID | Year | Title | Citations |
|---|---|---|---|
| 37690706 | 2024 | Upregulation of NOR-1 in calcified human vascular tissues: impact on osteogenic differentiation and calcification. | 2 |
| 37905340 | 2024 | RP11-495P10.1 promotes HCC cell proliferation by regulating reprogramming of glucose metabolism and acetylation of the NR4A3 promoter via the PDK1/PDH axis. | 0 |
| 38261254 | 2024 | Potential Roles of Nr4a3-Mediated Inflammation in Immunological and Neurological Diseases. | 0 |
| 38474099 | 2024 | Orphan Nuclear Receptor Family 4A (NR4A) Members NR4A2 and NR4A3 Selectively Modulate Elements of the Monocyte Response to Buffered Hypercapnia. | 0 |
| 38629274 | 2024 | Orphan Nuclear Receptor NR4A3 Promotes Vascular Calcification via Histone Lactylation. | 1 |
| 37690706 | 2024 | Upregulation of NOR-1 in calcified human vascular tissues: impact on osteogenic differentiation and calcification. | 2 |
| 37905340 | 2024 | RP11-495P10.1 promotes HCC cell proliferation by regulating reprogramming of glucose metabolism and acetylation of the NR4A3 promoter via the PDK1/PDH axis. | 0 |
| 38261254 | 2024 | Potential Roles of Nr4a3-Mediated Inflammation in Immunological and Neurological Diseases. | 0 |
| 38474099 | 2024 | Orphan Nuclear Receptor Family 4A (NR4A) Members NR4A2 and NR4A3 Selectively Modulate Elements of the Monocyte Response to Buffered Hypercapnia. | 0 |
| 38629274 | 2024 | Orphan Nuclear Receptor NR4A3 Promotes Vascular Calcification via Histone Lactylation. | 1 |
| 36469101 | 2023 | A minority of cases of acinic cell carcinoma of the salivary glands are driven by an NR4A2 rearrangement: the diagnostic utility of the assessment of NR4A2 and NR4A3 alterations in salivary gland tumors. | 0 |
| 36482877 | 2023 | Nuclear Receptor Subfamily 4A Signaling as a Key Disease Pathway of CD1c+ Dendritic Cell Dysregulation in Systemic Sclerosis. | 3 |
| 36524687 | 2023 | NR4A3 fusions characterize a distinctive peritoneal mesothelial neoplasm of uncertain biological potential with pure adenomatoid/microcystic morphology. | 1 |
| 36828363 | 2023 | NR4A3 Expression Is Consistently Absent in Acinic Cell Carcinomas of the Breast: A Potential Nosologic Shift. | 1 |
| 37389860 | 2023 | Loss of NOR-1 represses muscle metabolism through mTORC1-mediated signaling and mitochondrial gene expression in C2C12 myotubes. | 1 |
Citation
Goran Stenman
NR4A3 (nuclear receptor subfamily 4, group A, member 3)
Atlas Genet Cytogenet Oncol Haematol. 2003-07-01
Online version: http://atlasgeneticsoncology.org/gene/75
