NR4A3 (nuclear receptor subfamily 4, group A, member 3)

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NR4A3 (nuclear receptor subfamily 4, group A, member 3)

Identity

Other names TEC

NR4A3

NOR1

MINOR

CSMF

CHN

Hugo NR4A3

Location 9q22

DNA/RNA

Description spans about 46 kb; 9 exons

Transcription four transcript variants (6382 bp, 5635 bp, 4983bp and 2588 bp)

Protein

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

Entity Extraskeletal myxoid chondrosarcoma with t(9;22)(q22;q12).

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/Mutated Gene EWSR1-TEC

Abnormal Protein N-terminal transactivation domain of EWSR1 fused to the entire TEC protein.

Entity Extraskeletal myxoid chondrosarcoma with t(9;17)(q22;q11).

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/Mutated Gene TAF2N-TEC

Abnormal Protein N-terminal transactivation domain of TAF2N fused to the entire TEC protein.

Entity 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/Mutated Gene TCF12-TEC

Abnormal 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

External links

	Nomenclature
Hugo	NR4A3
GDB	NR4A3
Entrez_Gene	NR4A3 8013 nuclear receptor subfamily 4, group A, member 3
	Cards
Atlas	TECID75
GeneCards	NR4A3
Ensembl	NR4A3 [Search_View] ENSG00000119508 [Gene_View]
Genatlas	NR4A3
GeneLynx	NR4A3
eGenome	NR4A3
euGene	8013
	Genomic and cartography
GoldenPath	NR4A3 - 9q22 chr9:101623958-101668994 + 9q22 [Description] (hg18-Mar_2006)
Ensembl	NR4A3 - 9q22 [CytoView]
NCBI	Mapview
OMIM	Disease map [OMIM]
HomoloGene	NR4A3
	Gene and transcription
Genbank	AK226108 [ ENTREZ ]
Genbank	BX649089 [ ENTREZ ]
Genbank	D78579 [ ENTREZ ]
Genbank	D85241 [ ENTREZ ]
Genbank	D85242 [ ENTREZ ]
RefSeq	NM_006981 [ SRS ] NM_006981 [ ENTREZ ]
RefSeq	NM_173198 [ SRS ] NM_173198 [ ENTREZ ]
RefSeq	NM_173199 [ SRS ] NM_173199 [ ENTREZ ]
RefSeq	NM_173200 [ SRS ] NM_173200 [ ENTREZ ]
RefSeq	AC_000052 [ SRS ] AC_000052 [ ENTREZ ]
RefSeq	NC_000009 [ SRS ] NC_000009 [ ENTREZ ]
RefSeq	NT_008470 [ SRS ] NT_008470 [ ENTREZ ]
RefSeq	NW_924539 [ SRS ] NW_924539 [ ENTREZ ]
AceView	NR4A3 AceView - NCBI
Unigene	Hs.279522 [ SRS ] Hs.279522 [ NCBI ] HS279522 [ spliceNest ]
Fast-db	11913 (alternative variants)
	Protein : pattern, domain, 3D structure
SwissProt	Q92570 [ SRS] Q92570 [ EXPASY ] Q92570 [ INTERPRO ]
Prosite	PS00031 NUCLEAR_REC_DBD_1 [ SRS ] PS00031 NUCLEAR_REC_DBD_1 [ Expasy ]
Prosite	PS51030 NUCLEAR_REC_DBD_2 [ SRS ] PS51030 NUCLEAR_REC_DBD_2 [ Expasy ]
Interpro	IPR003072 NOR1_rcpt [ SRS ] IPR003072 NOR1_rcpt [ EBI ]
Interpro	IPR003070 Nuc_orph_rcpt [ SRS ] IPR003070 Nuc_orph_rcpt [ EBI ]
Interpro	IPR008946 Nucl_hormone_rcpt_ligand-bd [ SRS ] IPR008946 Nucl_hormone_rcpt_ligand-bd [ EBI ]
Interpro	IPR000536 Nucl_hrmn_rcpt_lig-bd_core [ SRS ] IPR000536 Nucl_hrmn_rcpt_lig-bd_core [ EBI ]
Interpro	IPR001723 Str_hrmn_rcpt [ SRS ] IPR001723 Str_hrmn_rcpt [ EBI ]
Interpro	IPR001628 Znf_hrmn_rcpt [ SRS ] IPR001628 Znf_hrmn_rcpt [ EBI ]
Interpro	IPR013088 Znf_NHR/GATA [ SRS ] IPR013088 Znf_NHR/GATA [ EBI ]
CluSTr	Q92570
Pfam	PF00104 Hormone_recep [ SRS ] PF00104 Hormone_recep [ Sanger ] pfam00104 [ NCBI-CDD ]
Pfam	PF00105 zf-C4 [ SRS ] PF00105 zf-C4 [ Sanger ] pfam00105 [ NCBI-CDD ]
Smart	SM00430 HOLI [EMBL]
Smart	SM00399 ZnF_C4 [EMBL]
Prodom	PD000035 Znf_C4steroid[INRA-Toulouse]
Prodom	Q92570 NR4A3_HUMAN [ Domain structure ] Q92570 NR4A3_HUMAN [ sequences sharing at least 1 domain ]
Blocks	Q92570
HPRD	02766
	Protein Interaction databases
DIP	Q92570
IntAct	Q92570
	Polymorphism : SNP, mutations, diseases
OMIM	600542 [ map ]
GENECLINICS	600542
SNP	NR4A3 [dbSNP-NCBI]
SNP	NM_006981 [SNP-NCI]
SNP	NM_173198 [SNP-NCI]
SNP	NM_173199 [SNP-NCI]
SNP	NM_173200 [SNP-NCI]
SNP	NR4A3 [GeneSNPs - Utah] NR4A3] [HGBASE - SRS]
HAPMAP	NR4A3 [HAPMAP]
COSMIC	NR4A3 [Somatic mutation (COSMIC-CGP-Sanger)]
TICdb	NR4A3 [Translocation breakpoints In Cancer]
HGMD	NR4A3
	General knowledge
Family Browser	NR4A3 [UCSC Family Browser]
SOURCE	NM_006981
SOURCE	NM_173198
SOURCE	NM_173199
SOURCE	NM_173200
SMD	Hs.279522
SAGE	Hs.279522
GO	transcription factor activity [Amigo] transcription factor activity
GO	steroid hormone receptor activity [Amigo] steroid hormone receptor activity
GO	ligand-dependent nuclear receptor activity [Amigo] ligand-dependent nuclear receptor activity
GO	thyroid hormone receptor activity [Amigo] thyroid hormone receptor activity
GO	binding [Amigo] binding
GO	nucleus [Amigo] nucleus
GO	nucleus [Amigo] nucleus
GO	transcription [Amigo] transcription
GO	regulation of transcription, DNA-dependent [Amigo] regulation of transcription, DNA-dependent
GO	biological_process [Amigo] biological_process
GO	zinc ion binding [Amigo] zinc ion binding
GO	sequence-specific DNA binding [Amigo] sequence-specific DNA binding
GO	metal ion binding [Amigo] metal ion binding
PubGene	NR4A3
TreeFam	NR4A3
CTD	8013 [Comparative ToxicoGenomics Database]
	Other databases
	Probes
Probe	NR4A3 Related clones (RZPD - Berlin)
	PubMed
PubMed	23 Pubmed reference(s) in LocusLink

Bibliography

Molecular cloning of a novel thyroid/steroid receptor superfamily gene from cultured rat neuronal cells.

Ohkura N, Hijikuro M, Yamamoto A, Miki K

Biochemical and biophysical research communications. 1994 ; 205 (3) : 1959-1965.

PMID 7811288

The isolation and characterization of MINOR, a novel mitogen-inducible nuclear orphan receptor.

Hedvat CV, Irving SG

Molecular endocrinology (Baltimore, Md.). 1995 ; 9 (12) : 1692-1700.

PMID 8614405

Oncogenic conversion of a novel orphan nuclear receptor by chromosome translocation.

Labelle Y, Zucman J, Stenman G, Kindblom LG, Knight J, Turc-Carel C, Dockhorn-Dworniczak B, Mandahl N, Desmaze C, Peter M

Human molecular genetics. 1995 ; 4 (12) : 2219-2226.

PMID 8634690

Fusion of the EWS gene to CHN, a member of the steroid/thyroid receptor gene superfamily, in a human myxoid chondrosarcoma.

Clark J, Benjamin H, Gill S, Sidhar S, Goodwin G, Crew J, Gusterson BA, Shipley J, Cooper CS

Oncogene. 1996 ; 12 (2) : 229-235.

PMID 8570200

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, Tariq M, Harris M, Coyne JD, Telford N, Varley JM

Oncogene. 1999 ; 18 (52) : 7599-7601.

PMID 10602520

The EWS/TEC fusion protein encoded by the t(9;22) chromosomal translocation in human chondrosarcomas is a highly potent transcriptional activator.

Labelle Y, Bussi��res J, Courjal F, Goldring MB

Oncogene. 1999 ; 18 (21) : 3303-3308.

PMID 10359536

Fusion of the EWS-related gene TAF2N to TEC in extraskeletal myxoid chondrosarcoma.

Sj��gren H, Meis-Kindblom J, Kindblom LG, Aman P, Stenman G

Cancer research. 1999 ; 59 (20) : 5064-5067.

PMID 10537274

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).

Sj��gren H, Wedell B, Meis-Kindblom JM, Kindblom LG, Stenman G

Cancer research. 2000 ; 60 (24) : 6832-6835.

PMID 11156374

The AF2 domain of the orphan nuclear receptor TEC is essential for the transcriptional activity of the oncogenic fusion protein EWS/TEC.

Maltais A, Filion C, Labelle Y

Cancer letters. 2002 ; 183 (1) : 87-94.

PMID 12049818

The EWS/NOR1 fusion gene product gains a novel activity affecting pre-mRNA splicing.

Ohkura N, Yaguchi H, Tsukada T, Yamaguchi K

The Journal of biological chemistry. 2002 ; 277 (1) : 535-543.

PMID 11673470

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, Filion C, Bridge JA, Ladanyi M, Goldring MB, Labelle Y

Cancer research. 2003 ; 63 (2) : 449-454.

PMID 12543801

REVIEW articles automatic search in PubMed

Last year publications automatic search in PubMed

Search in all EBI NCBI

Contributor(s)

Written 07-2003 Goran Stenman

Citation

This paper should be referenced as such :
Stenman G . NR4A3 (nuclear receptor subfamily 4, group A, member 3). Atlas Genet Cytogenet Oncol Haematol. July 2003 .
URL : http://AtlasGeneticsOncology.org/Genes/TECID75.html

© Atlas of Genetics and Cytogenetics in Oncology and Haematology
indexed on : Wed Jul 2 08:27:33 2008

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j.l.huret@chu-poitiers.fr.

Other names	TEC
	NR4A3
	NOR1
	MINOR
	CSMF
	CHN
Hugo	NR4A3
Location	9q22

Description	spans about 46 kb; 9 exons
Transcription	four transcript variants (6382 bp, 5635 bp, 4983bp and 2588 bp)

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.

Entity	Extraskeletal myxoid chondrosarcoma with t(9;22)(q22;q12).
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/Mutated Gene	EWSR1-TEC
Abnormal Protein	N-terminal transactivation domain of EWSR1 fused to the entire TEC protein.

Entity	Extraskeletal myxoid chondrosarcoma with t(9;17)(q22;q11).
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/Mutated Gene	TAF2N-TEC
Abnormal Protein	N-terminal transactivation domain of TAF2N fused to the entire TEC protein.

Entity	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/Mutated Gene	TCF12-TEC
Abnormal 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.

REVIEW articles	automatic search in PubMed
Last year publications	automatic search in PubMed