| | |  | | | | Record from Web of Science® | | | | | | |
| The RB1 gene is the target of chromosome 13 deletions in malignant fibrous histiocytoma |
|
|
| Author(s): Chibon F, Mairal A, Freneaux P, Terrier P, Coindre JM, Sastre X, Aurias A |
| Source: CANCER RESEARCH Volume: 60 Issue: 22 Pages: 6339-6345 Published: NOV 15 2000 |
| Times Cited: 19 References: 16 |
| Abstract: Forty-four malignant Fibrous histiocytomas (MFHs) were studied by comparative genomic hybridization. Among the observed imbalances, losses of the 13q14-q21 region were observed in almost all tumors (78%), suggesting that a gene localized in this region could act as a tumor suppressor gene and that its inactivation could be relevant for MFH oncogenesis and/or progression. We determined by CA repeat analyses a consensus region of deletion focusing on the RBI region. The RBI gene was then analyzed by protein truncation test, direct sequencing, fluorescence in situ hybridization, Southern blotting, and immunohistochemistry. RBI mutations and/or homozygous deletions were found in 7 of the 34 tumors analyzed (20%). Among the 35 tumors with comparative genomic hybridization imbalances analyzed by immunohistochemistry, 30 (86%) did not exhibit significant nuclear labeling. The high correlation between chromosome 13 losses and absence of RBI protein expression and the mutations detected strongly suggest that RBI gene inactivation is a pivotal event in MFH oncogenesis, Moreover, the observation of a high incidence of MFH in patients previously treated for hereditary retinoblastoma fits well this hypothesis. |
| Document Type: Article |
| Language: English |
| Reprint Address: Aurias, A (reprint author), Inst Curie, INSERM U509, Sect Rech, Lab Pathol Mol Canc, 26 Rue Ulm, F-75248 Paris 05, France |
Addresses:
1. Inst Curie, INSERM U509, Sect Rech, Lab Pathol Mol Canc, F-75248 Paris 05, France
2. Inst Curie, Dept Pathol, F-75248 Paris, France
3. Inst Gustave Roussy, Dept Pathol, F-94805 Villejuif, France
4. Inst Bergonie, Dept Pathol, F-33076 Bordeaux, France |
| Publisher: AMER ASSOC CANCER RESEARCH, PO BOX 11806, BIRMINGHAM, AL 35202 USA |
| Subject Category: Oncology |
| IDS Number: 375ZD |
| ISSN: 0008-5472 |
|
| | | | | | | | | Record from Web of Science® | | | | | | | | | |