Abstract
Cytogenetic abnormalities involving chromosome 14 band q32 are consistently observed in human T-cell tumors. Patients with ataxia-telangiectasia (AT) are especially prone to development of these tumors, which frequently carry either inversion inv(14)(q11;q32) or translocation t(14;14) (q11;q32) chromosomes. We have previously shown that the cytogenetic breakpoints of one t(14;14)(q11;q32) chromosome and two inv(14)(q11;q32) chromosomes in T-cell tumors from AT and non-AT patients join the T-cell receptor alpha chain locus, at chromosome band 14q11, with a region(s) at 14q32 centromeric of the immunoglobulin heavy chain variable region (VH) gene IGHV. We now show that these two inv(14) breakpoints are linked by 2.1 kb of germ-line 14q32 DNA and that the three breakpoints define, by in situ hybridization analysis, a single locus at chromosome band 14q32.1 located about 15-20 million base pairs on the centromeric side of the IGH locus. Sequence analysis of the 14q32.1 breakpoint regions indicates that abnormal recombination does not universally result from mistaken V-D-J joining (D = diversity region; J = joining region). Therefore, we invoke a tumor selection model to describe the role of the 14q32.1 locus in tumor development.
MeSH Terms
Base Sequence
Chromosome Mapping
Chromosomes, Human, Pair 14
Cloning, Molecular
Gene Rearrangement, T-Lymphocyte
Humans
Leukemia, T-Cell/genetics
Molecular Sequence Data
Multigene Family
Nucleic Acid Hybridization
Translocation, Genetic
Authors & Affiliations
4 authors, click to expand affiliations / ORCID
Mengle-Gaw L
Laboratory of Molecular Biology, University of Cambridge, England.
Albertson D G
Sherrington P D
Rabbitts T H
References (25)
25 references, click to expand
-
Untangling ataxia-telangiectasia.
Nature. 1981 Jan 22;289(5795):222-3
PMID: 7453822
-
Juxtaposition of the T-cell receptor alpha-chain locus (14q11) and a region (14q32) of potential importance in leukemogenesis by a 14;14 translocation in a patient with T-cell chronic lymphocytic leukemia and ataxia-telangiectasia.
Proc Natl Acad Sci U S A. 1988 Dec;85(23):9287-91
PMID: 3194425
-
Cloning in single-stranded bacteriophage as an aid to rapid DNA sequencing.
J Mol Biol. 1980 Oct 25;143(2):161-78
PMID: 6260957
-
A technique for radiolabeling DNA restriction endonuclease fragments to high specific activity.
Anal Biochem. 1983 Jul 1;132(1):6-13
PMID: 6312838
-
Cytogenetic studies on patients with chronic T cell leukemia/lymphoma.
Blood. 1984 May;63(5):1028-38
PMID: 6608968
-
Comparison of the hinge-coding segments in human immunoglobulin gamma heavy chain genes and the linkage of the gamma 2 and gamma 4 subclass genes.
EMBO J. 1982;1(4):403-7
PMID: 6329676
-
Localization of the ribosomal genes in Caenorhabditis elegans chromosomes by in situ hybridization using biotin-labeled probes.
EMBO J. 1984 Jun;3(6):1227-34
PMID: 6378619
-
Abnormalities of chromosome 14 at band 14q11 in Japanese patients with adult T-cell leukemia.
Cancer Genet Cytogenet. 1985 Jul;17(3):279-82
PMID: 2988754
-
Mapping muscle protein genes by in situ hybridization using biotin-labeled probes.
EMBO J. 1985 Oct;4(10):2493-8
PMID: 4054096
-
Fusion of an immunoglobulin variable gene and a T cell receptor constant gene in the chromosome 14 inversion associated with T cell tumors.
Cell. 1985 Dec;43(3 Pt 2):705-13
PMID: 3935328
-
Specific chromosome markers involved with chronic T lymphocyte tumors.
Cancer Genet Cytogenet. 1986 Mar 1;21(1):67-77
PMID: 3484672
-
New data on clonal anomalies of chromosome 14 in ataxia telangiectasia: tct(14;14) and inv(14).
Hum Genet. 1986 Jan;72(1):22-4
PMID: 3943860
-
Lymphocyte malignancy and chromosome 14: structural aberrations involving band q11.
Blood. 1986 Mar;67(3):704-9
PMID: 2936408
-
A chromosome 14 inversion in a T-cell lymphoma is caused by site-specific recombination between immunoglobulin and T-cell receptor loci.
Nature. 1986 Apr 10-16;320(6062):549-51
PMID: 3008004
-
Diversity and rearrangement of the human T cell rearranging gamma genes: nine germ-line variable genes belonging to two subgroups.
Cell. 1986 Apr 25;45(2):237-46
PMID: 2938743
-
The chromosome breakpoint at 14q32 in an ataxia telangiectasia t(14;14) T cell clone is different from the 14q32 breakpoint in Burkitts and an inv(14) T cell lymphoma.
Hum Genet. 1986 Jul;73(3):254-9
PMID: 3488254
-
Common mechanism of chromosome inversion in B- and T-cell tumors: relevance to lymphoid development.
Science. 1986 Oct 10;234(4773):197-200
PMID: 3092355
-
A common mechanism of chromosomal translocation in T- and B-cell neoplasia.
Science. 1986 Nov 21;234(4779):982-5
PMID: 3490692
-
Fragile sites limited to lymphocytes: molecular recombination and malignancy.
Cancer Genet Cytogenet. 1987 May;26(1):95-104
PMID: 3470137
-
The mechanism of chromosome 14 inversion in a human T cell lymphoma.
Cell. 1987 Jul 3;50(1):97-105
PMID: 3036367
-
A human chromosome 8 region with abnormalities in B cell, HTLV-I+ T cell and c-myc amplified tumours.
EMBO J. 1987 Jul;6(7):1959-65
PMID: 2820714
-
Human T-cell tumours containing chromosome 14 inversion or translocation with breakpoints proximal to immunoglobulin joining regions at 14q32.
EMBO J. 1987 Aug;6(8):2273-80
PMID: 3117531
-
The breakpoint of an inversion of chromosome 14 in a T-cell leukemia: sequences downstream of the immunoglobulin heavy chain locus are implicated in tumorigenesis.
Proc Natl Acad Sci U S A. 1987 Dec;84(24):9069-73
PMID: 3122210
-
Molecular analysis of a t(7;14)(q35;q32) chromosome translocation in a T cell leukemia of a patient with ataxia telangiectasia.
Cell. 1988 Apr 8;53(1):137-44
PMID: 3258192
-
Structure and multiplicity of genes for the human immunoglobulin heavy chain variable region.
Proc Natl Acad Sci U S A. 1980 Nov;77(11):6561-5
PMID: 6450418