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Question: Phenylketonuria (PKU) is a rare human hereditary disease. PKU is caused by a defect in the gene that helps create the enzyme needed to break down phenylalanine. Without the enzyme necessary to process phenylalanine, a dangerous buildup can develop when a person with PKU eats foods that contain protein or eats aspartame, an artificial sweetener. PKU is transmitted in an autosomal recessive manner, with simple Mendelian inheritance. In a pedigree, if a man has a sister with PKU, but his parents are unaffected with PKU, what is the probability that he is an unaffected carrier?
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