TLX3 (T-cell leukemia, homeobox protein 3)
2004-03-01 Roderick AF MacLeod  , Stefan Nagel   AffiliationDSMZ-Deutsche Sammlung von, Mikroorganismen und Zellkulturen, Inhoffenstr. 7B, D-38124 Braunschweig, Germany
Identity
HGNC
LOCATION
5q35.1
IMAGE

LEGEND
TLX3 (5q35.1): FISH with BAC clone ctb-45L16 (red) showing a breakpoint signal in the consensus breakpoint region split between der(5) and der(14) partners. Analysis was performed on the pediatric T-ALL cell line HPB-ALL (DSMZ ACC-483) which carries t(5;14)(q35.1;q32.2) as part of a complex 4-way rearrangement involving chromosomes 1 and 16. Normally (in the absence of secondary translocations) the breakpoints on both partners lie closely equidistant to the q-arm telomeres rendering the translocation cryptic and difficult to detect even with chromosome painting.
LOCUSID
ALIAS
HOX11L2,RNX
FUSION GENES
DNA/RNA

Description
3 exons on 2.23 kb
Transcription
In a centromeric --> telomeric orientation; 876 bp mRNA (coding)
Proteins

Description
291 amino acids, 31.8 kDa; contains one homeobox domain (residues 166-221)
Expression
Narrowly restricted to brain.
Localisation
Probably nuclear
Function
Murine Tlx-3 and Tlx-1 together sustain expression of Drg-11, and control development of somatic and visceral relay sensory neurons.
Homology
With homeobox genes, especially with those of the NK-like family.
Implicated in
Entity name
Disease
T-cell acute lymphocytic leukemia (T-ALL)
Prognosis
TLX3 expression may denote poor prognosis.
Cytogenetics
Hybrid gene
5 TLX3-3 BCL11B on der(14).
Oncogenesis
Ectopic expression in T-cells
Entity name
Disease
T-cell acute lymphocytic leukemia (T-ALL)
Cytogenetics
Apparent variant of t(5;14)
Hybrid gene
5 TLX3-TCRD on der(14)
Article Bibliography
| Pubmed ID | Last Year | Title | Authors |
|---|---|---|---|
| 12130513 | 2002 | HOX11L2 expression defines a clinical subtype of pediatric T-ALL associated with poor prognosis. | Ballerini P et al |
| 12970786 | 2003 | t(5;14)/HOX11L2-positive T-cell acute lymphoblastic leukemia. A collaborative study of the Groupe Français de Cytogénétique Hématologique (GFCH). | Berger R et al |
| 11587205 | 2001 | A new recurrent and specific cryptic translocation, t(5;14)(q35;q32), is associated with expression of the Hox11L2 gene in T acute lymphoblastic leukemia. | Bernard OA et al |
| 14504110 | 2004 | Clinical significance of HOX11L2 expression linked to t(5;14)(q35;q32), of HOX11 expression, and of SIL-TAL fusion in childhood T-cell malignancies: results of EORTC studies 58881 and 58951. | Cavé H et al |
| 11435716 | 2001 | Assignment of the HOX11L2 gene to human chromosome band 5q35.1 and of its murine homolog to mouse chromosome bands 11A4-A5 by in situ hybridization. | Cinti R et al |
| 12086890 | 2002 | Gene expression signatures define novel oncogenic pathways in T cell acute lymphoblastic leukemia. | Ferrando AA et al |
| 12399963 | 2002 | Disruption of the RanBP17/Hox11L2 region by recombination with the TCRdelta locus in acute lymphoblastic leukemias with t(5;14)(q34;q11). | Hansen-Hagge TE et al |
| 11435718 | 2001 | Assignment of the human homeobox 11-like 2 gene (HOX11L2) to chromosome 5q34-->q35 by radiation hybrid mapping. | Lee-Kirsch MA et al |
| 12661009 | 2003 | Activation of HOX11L2 by juxtaposition with 3'-BCL11B in an acute lymphoblastic leukemia cell line (HPB-ALL) with t(5;14)(q35;q32.2). | MacLeod RA et al |
| 12454747 | 2002 | High incidence of Hox11L2 expression in children with T-ALL. | Mauvieux L et al |
| 14500364 | 2003 | The cardiac homeobox gene NKX2-5 is deregulated by juxtaposition with BCL11B in pediatric T-ALL cell lines via a novel t(5;14)(q35.1;q32.2). | Nagel S et al |
Other Information
Locus ID:
NCBI: 30012
MIM: 604640
HGNC: 13532
Ensembl: ENSG00000164438
Variants:
dbSNP: 30012
ClinVar: 30012
TCGA: ENSG00000164438
COSMIC: TLX3
RNA/Proteins
| Gene ID | Transcript ID | Uniprot |
|---|---|---|
| ENSG00000164438 | ENST00000296921 | O43711 |
Expression (GTEx)
Pathways
| Pathway | Source | External ID |
|---|---|---|
| Transcriptional misregulation in cancer | KEGG | ko05202 |
| Transcriptional misregulation in cancer | KEGG | hsa05202 |
Protein levels (Protein atlas)
References
| Pubmed ID | Year | Title | Citations |
|---|---|---|---|
| 24571118 | 2016 | NKX2-5, SIL/TAL and TLX3/HOX11L2 expression in Egyptian pediatric T-cell acute lymphoblastic leukemia. | 2 |
| 26852660 | 2016 | Immunophenotyping with CD135 and CD117 predicts the FLT3, IL-7R and TLX3 gene mutations in childhood T-cell acute leukemia. | 3 |
| 24571118 | 2016 | NKX2-5, SIL/TAL and TLX3/HOX11L2 expression in Egyptian pediatric T-cell acute lymphoblastic leukemia. | 2 |
| 26852660 | 2016 | Immunophenotyping with CD135 and CD117 predicts the FLT3, IL-7R and TLX3 gene mutations in childhood T-cell acute leukemia. | 3 |
| 24997618 | 2015 | AMBRA1 and BECLIN 1 interplay in the crosstalk between autophagy and cell proliferation. | 0 |
| 24997618 | 2015 | AMBRA1 and BECLIN 1 interplay in the crosstalk between autophagy and cell proliferation. | 0 |
| 22366949 | 2012 | Reverse engineering of TLX oncogenic transcriptional networks identifies RUNX1 as tumor suppressor in T-ALL. | 80 |
| 22516263 | 2012 | TLX homeodomain oncogenes mediate T cell maturation arrest in T-ALL via interaction with ETS1 and suppression of TCRα gene expression. | 50 |
| 22366949 | 2012 | Reverse engineering of TLX oncogenic transcriptional networks identifies RUNX1 as tumor suppressor in T-ALL. | 80 |
| 22516263 | 2012 | TLX homeodomain oncogenes mediate T cell maturation arrest in T-ALL via interaction with ETS1 and suppression of TCRα gene expression. | 50 |
| 21225930 | 2011 | Successful treatment of a child with T/myeloid acute bilineal leukemia associated with TLX3/BCL11B fusion and 9q deletion. | 9 |
| 21374761 | 2011 | Wnt signaling promotes neuronal differentiation from mesenchymal stem cells through activation of Tlx3. | 44 |
| 21617853 | 2011 | Aberrant DNA methylation of T-cell leukemia, homeobox 3 modulates cisplatin sensitivity in bladder cancer. | 12 |
| 21225930 | 2011 | Successful treatment of a child with T/myeloid acute bilineal leukemia associated with TLX3/BCL11B fusion and 9q deletion. | 9 |
| 21374761 | 2011 | Wnt signaling promotes neuronal differentiation from mesenchymal stem cells through activation of Tlx3. | 44 |
Citation
Roderick AF MacLeod ; Stefan Nagel
TLX3 (T-cell leukemia, homeobox protein 3)
Atlas Genet Cytogenet Oncol Haematol. 2004-03-01
Online version: http://atlasgeneticsoncology.org/gene/398/tlx3-(t-cell-leukemia-homeobox-protein-3)


