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Cancer is an informal term for a diverse class of more than 100 distinct diseases marked by abnormal cell proliferation; white blood cells proliferate at an inappropriate rate, or other cell types form growths known as tumors (neoplasms). Benign tumors grow in only one place and do not invade other tissues. The cells of malignant tumors not only continue to proliferate but also invade nearby tissues and, by a process called metastasis, spread to distant parts of the body through blood or lymph vessels and start new centers of uncontrolled cell growth wherever they go. Cancers are generally divided into four groups, dependent on the type of cells originally involved. Two types of cancer cause overproduction of white blood cells. Leukemias are diseases that cause excessive production of leukocytes, which originate in the bone marrow. Lymphomas cause excessive production of lymphocytes, which originate in the lymph nodes and spleen. Sarcomas are tumors of tissue such as muscle, bone, and cartilage that arise from the embryological mesoderm. About 85% of cancers are carcinomas, tumors arising from epithelial tissue such as glands, breast, skin, and the linings of the urogenital, digestive, and respiratory systems. All cancers are genetic: They come about from alterations in genes that control cell growth. Most evidence indicates that cancers are clonal they arise from a single aberrant cell that then proliferates. Therefore, analyzing the causes of cancer comes down to trying to understand how one cell is changed, or transformed, from a
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Cancer-Family Syndromes
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In some cases, a predisposition for malignancies is inherited. When four thousand clinic registrants were interviewed, almost half reported virtually no family history of
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Tamarin: Principles of Genetics, Seventh Edition
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III. Molecular Genetics
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16. Gene Expression: Control in Eukaryotes
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The McGraw Hill Companies, 2001
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Cancer
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Table 16.3 A Small Sample of Chromosomal Rearrangements Associated
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with Speci c Cancers
Disease Burkitt s lymphoma Non-Hodgkin s lymphoma B-cell chronic lymphocytic leukemia Follicular lymphoma T/B-cell lymphoma Chronic myelogenous leukemia/ Acute lymphocytic leukemia Ewing s sarcoma Melanoma of soft parts Liposarcoma Chromosomal Rearrangement t(8; 14)* t(3; 4) t(11; 14) t(14; 18) Inversion, chromosome 14 t(9; 22) t(11; 22) t(12; 22) t(12; 16) Genes Affected c-MYC Laz3, BCL- 6 BCL-1, PRAD-1 BCL-2 TCR-a CABL FLI1, EWS ATF1, EWS CHOP, FUS
Reprinted with permission from Nature, Vol. 372, T. H. Rabbits. Copyright 1994 Macmillan Magazines Limited. * The notation of the form t(8; 14) indicates a t ranslocation between chromosomes 8 and 14.
G-banded chromosomes from a patient with chronic myelogenous leukemia showing a translocation of chromatin (arrows) from chromosome 22 to chromosome 9.
(Courtesy of Charles Rubin, M.D., University of Chicago, Department of Pediatric Hematology/Oncology.)
cancer, whereas about 7% reported that many family members had cancer. This 7% was considered cancer prone because three or more close relatives of the interviewed person had cancer.The interpretation of the study is that some families are predisposed toward cancer, but most are not, displacing the idea that everyone in the population has a uniform and low probability of developing cancer. Lending support to this interpretation are the cancer-family syndromes, in which family members seem to inherit a nonspeci c predisposition toward tumors of various types. At least twenty cancer-family syndromes are known. In gure 16.26, we see a pedigree for a cancer-family syndrome in which the predisposition for several different types of cancers, rather than a particular
Pedigree of a type I cancer-family syndrome. This is interpreted as the inheritance of the propensity toward cancer rather than the inheritance of any speci c type of cancer.
type of cancer, seems to be inherited. Women in this family get breast, colon, and pancreatic cancers, whereas men get colon, prostate, and pancreatic cancers.
Tumor-Suppressor Genes
There is a class of cancer-related genes called the tumorsuppressor genes (also called anti-oncogenes). These genes act by suppressing malignant growth. Mutations are recessive, and in the homozygous state, cancer
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