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Tumor suppressor gene inactivation. Tumor suppressor gene mutations are thought to initiate many forms of cancer. Both alleles of the tumor suppressor gene must be inactivated for a tumor to form. In familial cancer predisposition syndromes, a mutant allele of a suppressor gene is inherited and is present in every cell. However, tumors are not initiated until the second allele (inherited from the unaffected parent) is inactivated in a somatic cell. In nonfamilial cases, the inactivation of both alleles occurs through somatic mutations. The end result is the same: no functional suppressor gene, leading to tumor initiation. Source: Introduction to Cancer Genetics Chapters, The Online Metabolic and Molecular Bases of Inherited Disease Citation: Valle D, Beaudet AL, Vogelstein B, Kinzler KW, Antonarakis SE, Ballabio A, Gibson K, Mitchell G. The Online Metabolic and Molecular Bases of Inherited Disease; 2014 Available at: http://mhmedical.com/ Accessed: May 10, 2017 Copyright © 2017 McGraw-Hill Education. All rights reserved