Suppression of tumorigenicity in Wilms tumor by the p15.5-p14 region of chromosome 11

Science ◽  
1991 ◽  
Vol 254 (5029) ◽  
pp. 293-295 ◽  
Author(s):  
S. Dowdy ◽  
C. Fasching ◽  
D Araujo ◽  
K. Lai ◽  
E Livanos ◽  
...  
Keyword(s):  
Wilms Tumor ◽  
Chromosome 11

scholarly journals Loss of allelic heterozygosity at a second locus on chromosome 11 in sporadic Wilms' tumor cells.

1989 ◽  
Vol 9 (4) ◽  
pp. 1799-1803 ◽  
Author(s):  
A E Reeve ◽  
S A Sih ◽  
A M Raizis ◽  
A P Feinberg
Keyword(s):  
Mental Retardation ◽  
Tumor Cells ◽  
Germ Line ◽  
Wilms Tumor ◽  
Globin Gene ◽  
Allelic Loss ◽  
Chromosomal Band ◽  
Chromosome 11 ◽  
Gamma Globin ◽  
Wagr Syndrome

Children with associated Wilms' tumor, aniridia, genitourinary malformations, and mental retardation (WAGR syndrome) frequently have a cytogenetically visible germ line deletion of chromosomal band 11p13. In accordance with the Knudson hypothesis of two-hit carcinogenesis, the absence of this chromosomal band suggests that loss of both alleles of a gene at 11p13 causes Wilms' tumor. Consistent with this model, chromosomes from sporadically occurring Wilms' tumor cells frequently show loss of allelic heterozygosity at polymorphic 11p15 loci, and therefore it has been assumed that allelic loss extends proximally to include 11p13. We report here that in samples from five sporadic Wilms' tumors, allelic loss occurred distal to the WAGR locus on 11p13. In cells from one tumor, mitotic recombination occurred distal to the gamma-globin gene on 11p15.5. Thus, allelic loss in sporadic Wilms' tumor cells may involve a second locus on 11p.

scholarly journals Molecular analysis of chromosome 11 deletions in aniridia-Wilms tumor syndrome.

1985 ◽  
Vol 82 (24) ◽  
pp. 8592-8596 ◽  
Author(s):  
V. van Heyningen ◽  
P. A. Boyd ◽  
A. Seawright ◽  
J. M. Fletcher ◽  
J. A. Fantes ◽  
...  
Keyword(s):  
Molecular Analysis ◽  
Wilms Tumor ◽  
Chromosome 11

Mapping of Human Chromosome 11: Organization of Genes within the Wilms' Tumor Region of the Chromosome

1986 ◽  
Vol 51 (0) ◽  
pp. 837-841 ◽  
Author(s):  
D.E. Housman ◽  
T. Glaser ◽  
D.S. Gerhard ◽  
C. Jones ◽  
G.A.P. Bruns ◽  
...  
Keyword(s):  
Human Chromosome ◽  
Wilms Tumor ◽  
Chromosome 11 ◽  
Tumor Region

Localization of the oncogene c-Ha-ras1 outside the aniridia-Wilms' tumor-associated deletion of chromosome 11(del 11p13) using somatic cell hybrids

1985 ◽  
Vol 15 (1-2) ◽  
pp. 79-84 ◽  
Author(s):  
Ad Geurts van Kessel ◽  
Roel Nusse ◽  
Rosalyn Slater ◽  
Pedro Tetteroo ◽  
Anne Hagemeijer
Keyword(s):  
Somatic Cell ◽  
Wilms Tumor ◽  
Somatic Cell Hybrids ◽  
Chromosome 11 ◽  
Cell Hybrids

scholarly journals A sporadic case of congenital aniridia caused by pericentric inversion inv(11)(p13q14) associated with a 977 kb deletion in the 11p13 region

2020 ◽  
Vol 13 (S8) ◽  
Author(s):  
Tatyana A. Vasilyeva ◽  
Andrey V. Marakhonov ◽  
Marina E. Minzhenkova ◽  
Zhanna G. Markova ◽  
Nika V. Petrova ◽  
...  
Keyword(s):  
General Population ◽  
Pericentric Inversion ◽  
De Novo ◽  
Wilms Tumor ◽  
Normal Karyotype ◽  
Paternal Allele ◽  
De Novo Mutations ◽  
Chromosome 11 ◽  
Dna Diagnosis ◽  
Congenital Aniridia

Abstract Background Because of the significant occurrence of “WAGR-region” deletions among de novo mutations detected in congenital aniridia, DNA diagnosis is critical for all sporadic cases of aniridia due to its help in making an early diagnosis of WAGR syndrome. Standard cytogenetic karyotype study is a necessary step of molecular diagnostics in patients with deletions and in the patients’ parents as it reveals complex chromosomal rearrangements and the risk of having another affected child, as well as to provide prenatal and/or preimplantation diagnostics. Case presentation DNA samples were obtained from the proband (a 2-year-old boy) and his two healthy parents. Molecular analysis revealed a 977.065 kb deletion that removed loci of the ELP4, PAX6, and RCN1 genes but did not affect the coding sequence of the WT1 gene. The deletion occurred de novo on the paternal allele. The patient had normal karyotype 46,XY and a de novo pericentric inversion of chromosome 11, inv(11)(p13q14). Conclusions We confirmed the diagnosis of congenital aniridia at the molecular level. For the patient, the risk of developing Wilms’ tumor is similar to that in the general population. The recurrence risk for sibs in the family is low, but considering the possibility of gonadal mosaicism, it is higher than in the general population.

Isolation and characterization of a zinc finger polypeptide gene at the human chromosome 11 Wilms' tumor locus

Cell ◽  
1990 ◽  
Vol 60 (3) ◽  
pp. 509-520 ◽  
Author(s):  
Katherine M. Call ◽  
Tom Glaser ◽  
Caryn Y. Ito ◽  
Alan J. Buckler ◽  
Jerry Pelletier ◽  
...  
Keyword(s):  
Human Chromosome ◽  
Zinc Finger ◽  
Wilms Tumor ◽  
Chromosome 11 ◽  
Isolation And Characterization

Human chromosome 11 suppresses the tumorigenicity of adenovirus transformed baby rat kidney cells: Involvement of the wilms' tumor 1 gene

1995 ◽  
Vol 63 (1) ◽  
pp. 76-85 ◽  
Author(s):  
A. L. Menke ◽  
R. C. A. Van Ham ◽  
E. Sonneveld ◽  
A. Shvarts ◽  
E. J. Stanbridge ◽  
...  
Keyword(s):  
Human Chromosome ◽  
Wilms Tumor ◽  
Rat Kidney ◽  
Kidney Cells ◽  
Chromosome 11 ◽  
Wilms Tumor 1

Suppression of tumorigenicity in Wilms tumor by the p15.5-p14 region of chromosome 11

Science ◽  
1991 ◽  
Vol 254 (5029) ◽  
pp. 293-295
Author(s):  
SF Dowdy ◽  
CL Fasching ◽  
D Araujo ◽  
KM Lai ◽  
E Livanos ◽  
...  
Keyword(s):  
Tumor Cell ◽  
Cell Line ◽  
Nude Mice ◽  
Wilms Tumor ◽  
Genetic Locus ◽  
Tumor Cell Line ◽  
Tumor Formation ◽  
The Other ◽  
Chromosome 11

Wilms tumor has been associated with genomic alterations at both the 11p13 and 11p15 regions. To differentiate between the involvement of these two loci, a chromosome 11 was constructed that had one or the other region deleted, and this chromosome was introduced into the tumorigenic Wilms tumor cell line G401. When assayed for tumor-forming activity in nude mice, the 11p13-deleted, but not the 11p15.5-p14.1-deleted chromosome, retained its ability to suppress tumor formation. These results provide in vivo functional evidence for the existence of a second genetic locus (WT2) involved in suppressing the tumorigenic phenotype of Wilms tumor.

Wilms' tumor: status report, 1990. By the National Wilms' Tumor Study Committee.

1991 ◽  
Vol 9 (5) ◽  
pp. 877-887 ◽  
Keyword(s):  
Risk Factors ◽  
Wilms Tumor ◽  
Combined Modality Therapy ◽  
Genetic Research ◽  
Status Report ◽  
Tumor Type ◽  
Chromosome 11 ◽  
Hair Dyes ◽  
Tumor Study ◽  
Iatrogenic Complications

Rapid advances in the understanding of Wilms' tumor (WT) and its management are being made both in the laboratory and the clinic. Molecular genetic research has implicated loss of a tumor suppressor gene on the short arm of chromosome 11 as one of the pathways responsible for the development of the neoplasm. Preconception maternal (hair dyes) and paternal (occupation) exposures to environmental agents have been the subject of epidemiologic studies of possible risk factors. Histopathologic analyses have identified several different and less common tumor types among those previously aggregated under the WT rubric. WT itself has been subdivided into the so-called favorable histology (FH) and anaplastic forms, the prognosis being worse for the latter. Clinical research has standardized management by surgery, chemotherapy, and radiation therapy (RT) and furthered the identification of risk factors. Patients can now be stratified according to tumor type and stage, and the intensity of treatment modulated accordingly; eg, RT at low doses is used in only 25% of National Wilms' Tumor Study (NWTS) patients without distant metastases. Before the NWTS, it had been given to almost all and at higher doses. Chemotherapy, whether given pre- or postoperatively, is based on dactinomycin and vincristine with Adriamycin [( ADR] doxorubicin; Adria Laboratories, Columbus, OH) added for high-risk patients. The currently used NWTS combined modality therapy for WT patients has dramatically improved survival rates; 95% now are alive 2 years after treatment. Remaining questions are the identification of the late effects of the treatments used and the further refinement of therapy to reduce iatrogenic complications to a minimum.

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