XL CRLF2 BA

Break Apart Probe

Order Number
D-5130-100-OG
Package Size
100 µl (10 Tests)
Labels
  
Chromosomes
XY
Regulatory Status
IVDD

IVDR Certification

MetaSystems Probes has already certified a large part of its portfolio, according to IVDR.

Discover all IVDR-certified products

For organizational reasons, we currently provide only the IVDD product.

Product Description

XL CRLF2 BA

XL CRLF2 BA consists of an orange-labeled probe hybridizing proximal to the CRLF2 gene region at Xp22.33 and Yp11.32 and a green-labeled probe hybridizing distal to the CRLF2 gene region at Xp22.33 and Yp11.32.

Probe maps are created in accordance with the intended purpose of the product. Solid colored bars do not necessarily indicate that the probe fully covers the indicated genomic region. Therefore, caution is advised when interpreting results generated through off-label use. Probe map details based on UCSC Genome Browser GRCh37/hg19. Map components not to scale. Further information is available on request.

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Clinical Details

Acute lymphoblastic leukemia (ALL) is the most common malignancy in children (prevalence of approximately 1:1500). Children with Down syndrome have a 10- to 20-fold increased risk of developing acute leukemia. B-Cell dependent BCR-ABL1-like ALL, also known as Philadelphia chromosome (Ph)-like ALL, is a high-risk subset with a gene expression profile which shares significant overlap with that of Ph-positive (Ph+) ALL, but lacking the BCR-ABL1 fusion. In 2017, the WHO recognized BCR-ABL1-like ALL as a new entity.
Chromosomal rearrangements resulting in the overexpression of cytokine receptor like factor 2 (CRLF2) can be found in up to 50% of BCR-ABL1-like ALL cases. The CRLF2 gene is located in the pseudoautosomal region 1 (PAR1) of the X and the Y chromosome. Three genetic key mechanisms regarding CRLF2 and ALL are known. Firstly, the CRLF2 gene is placed under the control of the IGH enhancer. Translocations of the type t(X;14) or t(Y;14) are the genetic basis for this aberration. Secondly, fusion of CRLF2 to CSF2RA, a further PAR1 gene, has been described. Thirdly, cryptic interstitial deletions juxtaposing the initial non-coding exon of the purinergic receptor P2Y8 (P2RY8) and CRLF2 have been shown. The resulting P2RY8-CRLF2 fusion under the control of the P2RY8 promoter is strongly transcribed in lymphoid cells. CRLF2 rearrangements result in increased protein levels, which initiate significantly enhanced JAK/STAT signaling, whereby disproportionate JAK and subsequent STAT5 activation induces strongly enhanced B-cell activation and proliferation.

XL CRLF2 BA detects chromosomal aberrations resulting from CRLF2 gene rearrangements (deletions and translocations).
XL P2RY8 del (D-5150-100-OG) can be used as an additional tool in order to detect the presence of the P2RY8-CRLF2 fusion gene.

Clinical Applications

  • Acute Lymphoblastic Leukemia (ALL)
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Images

XL CRLF2 BA

XL CRLF2 BA hybridized to bone marrow cells. Two aberrant cells of a patient with a gonosomal constellation of XXY are shown. Two normal CRLF2 loci were observed indicated by two fusion signals. The separate green signal indicates a deletion proximal of CRLF2.

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Expected Patterns

Expected Pattern 1

Normal Cell:
Two green-orange colocalization/fusion signals (2GO).

Expected Pattern 2

Aberrant Cell (typical results):
One green-orange colocalization/fusion signal (1GO), one separate green (1G) and orange (1O) signal each resulting from a chromosome break in the relevant locus.

Expected Pattern 3

Aberrant Cell (typical results):
One green-orange (1GO) colocalization/fusion signal and one green (1G) signal resulting from the loss of one orange signal.

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Literature

  • Roll and Reuther (2010) Cancer Res 70:7347-7352
  • Yoda et al (2010) Proc Natl Acad Sci 107:252-257
  • Tasian et al (2017) Blood 130:2064-2072

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News

IVDR Certification

MetaSystems Probes has received IVDR certification for our initial 26 fluorescence in situ hybridization (FISH) probes from the notified body, BSI. Achieving this milestone was not without its challenges, and we are delighted to have accomplished IVDR certification for this probe set at an early stage.

IVDR Certification

New Probes Catalog is Online!

 

MetaSystems Probes is pleased to announce the new catalog including new and innovative products and supporting information. A PDF version of the new catalog is available for download here.

New Probes Catalog is Online!