There are three broad categories of cancer-related genes: proto-oncogenes, tumor suppressor genes, and DNA repair/stability genes. Distinguish between these three groups, then indicate which you think RB1 belongs to.

Human Anatomy & Physiology (11th Edition)
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Retinoblastoma: The Hits Just Keep Coming

1.a There are three broad categories of cancer-related genes: proto-oncogenes, tumor suppressor genes, and DNA repair/stability genes. Distinguish between these three groups, then indicate which you think RB1 belongs to.

b. In 1971, Alfred Knudson noticed that children like Kay, who have retinoblastoma in both eyes (bilateral), presented at earlier ages than those who had unilateral disease. To explain these different disease kinetics, he proposed the “two-hit hypothesis.” What does this hypothesis propose?

c. Consider Dr. Whitt’s comments about the relationship between the wild-type and mutant RB1 alleles and what you now know about Knudson’s two-hit hypothesis. Do these ideas seem to confirm or contradict your earlier prediction (based on the pedigree) about whether the mutant RB1 allele is dominant or recessive to the wild-type allele? Explain.

d. Do you think that Kay (and Julie) will be more likely to develop other cancers later in life?

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