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| Status |
Public on Jul 18, 2009 |
| Title |
Deregulated expression of cytokine receptor gene, CRLF2, is involved in lymphoid transformation in BCP-ALL |
| Organism |
Homo sapiens |
| Experiment type |
Genome variation profiling by genome tiling array
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| Summary |
Deregulated expression of cytokine receptor gene, CRLF2, is involved in lymphoid transformation in B-cell precursor acute lymphoblastic leukemia
We report two novel, cryptic chromosomal abnormalities in precursor B-cell acute lymphoblastic leukemia (BCP-ALL): a translocation, either t(X;14)(p22;q32) or t(Y;14)(p11;q32), in 33 patients and an interstitial deletion, either del(X)(p22.33p22.33) or del(Y)(p11.32p11.32), in 64 patients, involving the pseudoautosomal region (PAR1) of the sex chromosomes. The incidence of these abnormalities was 5% in childhood ALL (0.8% with the translocation, 4.2% with the deletion). Patients with the translocation were older (median age 16 years), whilst the patients with the deletion were younger (median age 4 years). The two abnormalities result in deregulated expression of the cytokine receptor, cytokine receptor-like factor 2, CRLF2 (also known as thymic stromal-derived lymphopoietin receptor, TSLPR). Over-expression of CRLF2 was associated with activation of the JAK-STAT pathway in cell lines and transduced primary B-cell progenitors, sustaining their proliferation and indicating a causal role of CRLF2 over-expression in lymphoid transformation. In Down Syndrome (DS) ALL and two non DS BCP-ALL cell lines, CRLF2 deregulation was associated with mutations of the JAK2 pseudokinase domain suggesting oncogenic cooperation, as well as highlighting a link between non DS ALL and JAK2 mutations.
Keyword(s): Global copy number analysis using Agilent oligonucleotide arrays
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| Overall design |
DNA copy number analysis of 16 acute lymphoblastic leukaemia samples (13 diagnostic, 1 diagnostic and relapse pair and 2 cell-lines) was performed using Agilent 244K and 105K custom microarrays. These samples were hybridised against gender matched reference DNA.
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| Contributor(s) |
Rand V |
| Citation(s) |
19641190 |
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| Submission date |
Jul 17, 2009 |
| Last update date |
Oct 26, 2016 |
| Contact name |
Vikki Rand |
| E-mail(s) |
[email protected]
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| Organization name |
Newcastle University
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| Department |
Northern Institute for Cancer Research
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| Lab |
Lymphoma Genomics
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| Street address |
Paul O'Gorman Building, Newcastle University
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| City |
Newcastle Upon Tyne |
| State/province |
Tyne and Wear |
| ZIP/Postal code |
NE12 4HH |
| Country |
United Kingdom |
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| Platforms (2) |
| GPL4091 |
Agilent-014693 Human Genome CGH Microarray 244A (Feature number version) |
| GPL8876 |
Agilent custom 105K array (Agilent-019507) |
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| Samples (16)
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| Relations |
| BioProject |
PRJNA119765 |
| Supplementary file |
Size |
Download |
File type/resource |
| GSE17165_RAW.tar |
880.5 Mb |
(http)(custom) |
TAR (of TXT) |
| Processed data included within Sample table |
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