PMID- 17011977
OWN - NLM
STAT- MEDLINE
DCOM- 20061218
LR  - 20120625
IS  - 0165-4608 (Print)
IS  - 0165-4608 (Linking)
VI  - 170
IP  - 2
DP  - 2006 Oct 15
TI  - Deletion size characterization of der(9) deletions in Philadelphia-positive 
      chronic myeloid leukemia.
PG  - 89-92
AB  - About 95% of the CML patients with chronic myeloid leukemia (CML) have a 
      Philadelphia chromosome resulting from a reciprocal translocation between bands 
      9q34 and 22q11.2 that juxtaposes the 3' region of the ABL gene to the 5' region 
      of BCR. Over the past few years, submicroscopic deletions due to the loss of 
      sequences proximal to chromosome 9 breakpoint or distal to chromosome 22 
      breakpoint have been found using fluorescence in situ hybridization (FISH). Among 
      150 CML bone marrow samples analyzed by molecular cytogenetics in our laboratory, 
      11 had a der(9) deletion detectable by FISH (deletion of the 5'ABL region and 
      3'BCR region in 10 samples and deletion of the 5'ABL region solely in 1 sample). 
      To delineate the size of the deletions, FISH mapping was performed using 22 
      bacterial artificial chromosomes (BACs), 11 on either side of the breakpoints, 
      the mean distance between BACs being 0.5 Mb. The deletion size of the 5'ABL 
      region on the der(9) extended from 2 to 5 Mb, the minimal deletion size being 
      localized between BACs RP11-101E3 and RP11-83J21. In two patients, the deletion 
      size of the 3'BCR region was about 500 kb (between RP11-80O7 and RP11-681C06). 
      The poor prognosis associated with these deletions was postulated by several 
      workers to be explained by haploinsufficiency of a tumor suppressor gene. 
      However, in our cases, the hypothetical deletion of one or more tumor suppressor 
      genes is not sufficient to explain the poor response to interferon therapy, but 
      the good response to imatinib treatment. We think that there could be one or more 
      genes coding for interferon receptors or for proteins acting directly or 
      indirectly with these receptors in the deleted regions.
FAU - Douet-Guilbert, Nathalie
AU  - Douet-Guilbert N
AD  - Laboratory of Histology, Embryology and Cytogenetics, Faculty of Medicine and 
      Health Sciences, Universite de Bretagne Occidentale (UBO), Brest, France.
FAU - Morel, Frederic
AU  - Morel F
FAU - Quemener, Sylvia
AU  - Quemener S
FAU - Maguer, Aurelie
AU  - Maguer A
FAU - Le Bris, Marie-Josee
AU  - Le Bris MJ
FAU - Morice, Patrick
AU  - Morice P
FAU - Berthou, Christian
AU  - Berthou C
FAU - De Braekeleer, Marc
AU  - De Braekeleer M
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Cancer Genet Cytogenet
JT  - Cancer genetics and cytogenetics
JID - 7909240
RN  - EC 2.7.10.2 (Proto-Oncogene Proteins c-abl)
RN  - EC 2.7.11.1 (BCR protein, human)
RN  - EC 2.7.11.1 (Proto-Oncogene Proteins c-bcr)
SB  - IM
MH  - Chromosomes, Artificial, Bacterial
MH  - *Chromosomes, Human, Pair 9
MH  - Humans
MH  - In Situ Hybridization, Fluorescence
MH  - Leukemia, Myelogenous, Chronic, BCR-ABL Positive/*genetics
MH  - Proto-Oncogene Proteins c-abl/genetics
MH  - Proto-Oncogene Proteins c-bcr/genetics
MH  - *Sequence Deletion
EDAT- 2006/10/03 09:00
MHDA- 2006/12/19 09:00
CRDT- 2006/10/03 09:00
PHST- 2006/05/30 00:00 [received]
PHST- 2006/06/14 00:00 [accepted]
PHST- 2006/10/03 09:00 [pubmed]
PHST- 2006/12/19 09:00 [medline]
PHST- 2006/10/03 09:00 [entrez]
AID - S0165-4608(06)00403-1 [pii]
AID - 10.1016/j.cancergencyto.2006.06.006 [doi]
PST - ppublish
SO  - Cancer Genet Cytogenet. 2006 Oct 15;170(2):89-92. doi: 
      10.1016/j.cancergencyto.2006.06.006.